Publications by authors named "Jeffrey P Blount"

152 Publications

Epilepsy surgery in infants up to 3 months of age: Safety, feasibility, and outcomes: A multicenter, multinational study.

Epilepsia 2021 Jun 14. Epub 2021 Jun 14.

Department of Neurosurgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.

Objective: Drug-resistant epilepsy (DRE) during the first few months of life is challenging and necessitates aggressive treatment, including surgery. Because the most common causes of DRE in infancy are related to extensive developmental anomalies, surgery often entails extensive tissue resections or disconnection. The literature on "ultra-early" epilepsy surgery is sparse, with limited data concerning efficacy controlling the seizures, and safety. The current study's goal is to review the safety and efficacy of ultra-early epilepsy surgery performed before the age of 3 months.

Methods: To achieve a large sample size and external validity, a multinational, multicenter retrospective study was performed, focusing on epilepsy surgery for infants younger than 3 months of age. Collected data included epilepsy characteristics, surgical details, epilepsy outcome, and complications.

Results: Sixty-four patients underwent 69 surgeries before the age of 3 months. The most common pathologies were cortical dysplasia (28), hemimegalencephaly (17), and tubers (5). The most common procedures were hemispheric surgeries (48 procedures). Two cases were intentionally staged, and one was unexpectedly aborted. Nearly all patients received blood products. There were no perioperative deaths and no major unexpected permanent morbidities. Twenty-five percent of patients undergoing hemispheric surgeries developed hydrocephalus. Excellent epilepsy outcome (International League Against Epilepsy [ILAE] grade I) was achieved in 66% of cases over a median follow-up of 41 months (19-104 interquartile range [IQR]). The number of antiseizure medications was significantly reduced (median 2 drugs, 1-3 IQR, p < .0001). Outcome was not significantly associated with the type of surgery (hemispheric or more limited resections).

Significance: Epilepsy surgery during the first few months of life is associated with excellent seizure control, and when performed by highly experienced teams, is not associated with more permanent morbidity than surgery in older infants. Thus surgical treatment should not be postponed to treat DRE in very young infants based on their age.
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http://dx.doi.org/10.1111/epi.16959DOI Listing
June 2021

Tethered spinal cord among individuals with myelomeningocele: an analysis of the National Spina Bifida Patient Registry.

J Neurosurg Pediatr 2021 May 7:1-7. Epub 2021 May 7.

8Developmental Behavioral Pediatrics, Seattle Children's Hospital and University of Washington College of Medicine, Seattle, Washington.

Objective: The aims of this study were to review the National Spina Bifida Patient Registry (NSBPR) data set to study the rates of tethered spinal cord release (TCR) among patients with myelomeningocele and variability between centers, to compare TCR rates between males and females, and to study the relationships between TCR rates and other condition-specific characteristics.

Methods: The NSBPR registry was queried to identify all patients with myelomeningocele. TCR rates were calculated over time using survival analyses; rates between centers and between males and females were compared. Cox proportional hazards models were constructed to identify relationships between TCR rates and sex, functional lesion level, ambulation status, treated hydrocephalus, and prior Chiari decompression.

Results: Of 6339 patients with information about their operations, 1366 (21.5%) underwent TCR, with significant variability between centers. The majority (75.8%) underwent a single TCR. The annual TCR rate was linear between birth and 13 years (1.8%/year) but declined sharply from 14 to 21 years (0.7%/year). There was no period of time at which the TCR rate accelerated. There were no significant differences in TCR rates between males and females. TCR rate was not related to functional lesion level but was lower among nonambulators compared with community ambulators (p = 0.005) and among those with treated hydrocephalus (HR 0.30, p < 0.001), and higher among those having prior Chiari decompression (HR 1.71, p < 0.001).

Conclusions: These results extend the results of prior single-institution studies, demonstrate significant treatment variability between institutions, and challenge the traditional concept that tethering is related to spinal cord stretching due to spinal growth.
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http://dx.doi.org/10.3171/2020.12.PEDS20868DOI Listing
May 2021

ACR Appropriateness Criteria® Seizures-Child.

J Am Coll Radiol 2021 May;18(5S):S199-S211

Specialty Chair, Riley Hospital for Children Indiana University, Indianapolis, Indiana.

In children, seizures represent an extremely heterogeneous group of medical conditions ranging from benign cases, such as a simple febrile seizure, to life-threatening situations, such as status epilepticus. Underlying causes of seizures also represent a wide range of pathologies from idiopathic cases, usually genetic, to a variety of acute and chronic intracranial or systemic abnormalities. This document discusses appropriate utilization of neuroimaging tests in a child with seizures. The clinical scenarios in this document take into consideration different circumstances at the time of a child's presentation including the patient's age, precipitating event (if any), and clinical and electroencephalogram findings and include neonatal seizures, simple and complex febrile seizures, post-traumatic seizures, focal seizures, primary generalized seizures in a neurologically normal child, and generalized seizures in neurologically abnormal child. This practical approach aims to guide clinicians in clinical decision-making and to help identify efficient and appropriate imaging workup. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.
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http://dx.doi.org/10.1016/j.jacr.2021.02.020DOI Listing
May 2021

The International Society for Pediatric Neurosurgery resolution on mandatory folic acid fortification of staple foods for prevention of spina bifida and anencephaly and associated disability and child mortality.

Childs Nerv Syst 2021 Jun 3;37(6):1809-1812. Epub 2021 May 3.

Department of Neurosurgery, Hospital Nacional de Ninos ¨Dr. Carlos Saenz Herrera¨, Paseo Colon y Calle 20 Sur, San Jose, 10103, Costa Rica.

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http://dx.doi.org/10.1007/s00381-021-05191-yDOI Listing
June 2021

Imaging characteristics associated with surgery in Chiari malformation type I.

J Neurosurg Pediatr 2021 Apr 23:1-9. Epub 2021 Apr 23.

1Division of Pediatric Neurosurgery, Department of Neurosurgery; and.

Objective: In Chiari malformation type I (CM-I), a variety of imaging findings have been purported to be important; however, results have been inconclusive, inconsistent, or not replicated in independent studies. The purpose of this study was to report imaging characteristics for a large cohort of patients with CM-I and identify the imaging findings associated with surgical decompression.

Methods: Patients were identified using ICD-9 codes for CM-I for the period from 1996 to 2017. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, or 3) did not have available preoperative MRI. Retrospective chart review was performed to collect demographic and clinical data. Imaging parameters were measured according to the Chiari I Malformation Common Data Elements.

Results: A total of 731 patients were included for analysis, having a mean follow-up duration of 25.5 months. The mean age at presentation was 8.5 years. The mean tonsil position was 11.4 mm below the foramen magnum, and 62.8% of patients had a pegged tonsil shape. Two hundred patients (27.4%) underwent surgery for life-dominating tussive headache, lower cranial nerve dysfunction, syrinx, and/or brainstem dysfunction. Surgical treatment was associated with a syrinx (OR 20.4, 95% CI 12.3-33.3, p < 0.0001), CM-1.5 (OR 1.797, 95% CI 1.08-2.98, p = 0.023), lower tonsil position (OR 1.130, 95% CI 1.08-1.18, p < 0.0001), and congenital fusion of cervical vertebrae (OR 5.473, 95% CI 1.08-27.8, p = 0.040). Among patients with benign CM-I, tonsil position was statistically significantly associated with future surgery.

Conclusions: Comprehensive imaging characteristics for a large cohort of patients with CM-I are reported. Analysis showed that a lower tonsillar position, a syrinx, and CM-1.5 were associated with undergoing posterior fossa decompression. This study demonstrates the importance of considering imaging findings in the context of patient symptomatology.
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http://dx.doi.org/10.3171/2020.9.PEDS20347DOI Listing
April 2021

Neurosurgical procedures for children with myelomeningocele after fetal or postnatal surgery: a comparative effectiveness study.

Dev Med Child Neurol 2021 Jan 2. Epub 2021 Jan 2.

Division of Urology, Department of Surgery, Duke University Medical Center, Durham, NC, USA.

Aim: To compare the frequencies of neurosurgical procedures to treat comorbid conditions of myelomeningocele in patients who underwent fetal surgery versus postnatal surgery for closure of the placode.

Method: By utilizing the National Spina Bifida Patient Registry in a comparative effectiveness study, 298 fetal surgery patients were matched by birthdate (±3mo) and spina bifida clinic site with one to three postnatal surgery patients (n=648). Histories were obtained by record review on enrollment and yearly subsequently. Multivariable Poisson regression was used to compare frequencies of procedures between cohorts, with adjustments for sex, ethnicity, insurance status, spinal segmental level of motor function, age at last visit recorded in the Registry, and, for shunt revision in shunted patients, age at cerebrospinal fluid (CSF) diversion.

Results: The median age at last visit was 4 years. In fully adjusted analyses in patients aged at least 12 months old, fetal surgery was associated with decreased frequency of CSF diversion for hydrocephalus by ventriculoperitoneal shunt insertion or endoscopic third ventriculostomy compared with postnatal surgery (46% vs 79%; incidence rate ratio=0.61; 95% confidence interval [CI] 0.53-0.71; p<0.01). Over all ages, fetal surgery was associated with decreased frequency of Chiari decompression for brainstem dysfunction (3% vs 7%; incidence rate ratio=0.41; 95% CI 0.19-0.88; p=0.02). Also over all ages, differences were not significant in frequencies of shunt revision in shunted patients (53% vs 55%; incidence rate ratio=0.87; 95% CI 0.69-1.11; p=0.27), nor tethered cord release for acquired spinal cord dysfunction (18% vs 16%; incidence rate ratio=1.11; 95% CI 0.84-1.47; p=0.46).

Interpretation: Even with the variations inherent in clinical practice, fetal surgery was associated with lower frequencies of CSF diversion and of Chiari decompression, independent of covariates.
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http://dx.doi.org/10.1111/dmcn.14792DOI Listing
January 2021

Prevalence of Sleep Disordered Breathing in Children With Myelomeningocele.

Neurosurgery 2021 03;88(4):785-790

Division of Pediatric Neurosurgery, Department of Neurosurgery, University of Alabama at Birmingham, Birmingham, Alabama.

Background: Retrospective studies have shown high rates of sleep disordered breathing in children with myelomeningocele. However, most patients included in those studies underwent polysomnography because of symptoms, so the prevalence of sleep disordered breathing in this population is unknown.

Objective: To determine the prevalence of sleep disordered breathing in children with myelomeningocele using screening polysomnography.

Methods: In this cross-sectional study, all children with myelomeningocele seen in a multi-disciplinary spina bifida clinic between 2016 and 2020 were referred for polysomnography regardless of clinical symptoms. Included children had not previously undergone polysomnography. The primary outcome for this study was presence of sleep disordered breathing, defined as Apnea-Hypopnea Index (AHI, number of apnea or hypopnea events per hour of sleep) greater than 2.5. Clinical and demographic variables relevant to myelomeningocele were also prospectively collected and tested for association with presence of sleep apnea.

Results: A total of 117 participants underwent polysomnography (age 1 mo to 21 yr, 49% male). The majority were white, non-Hispanic. Median AHI was 1.9 (interquartile range 0.6-4.2). A total of 49 children had AHI 2.5 or greater, yielding a sleep disordered breathing prevalence of 42% (95% CI 33%-51%). In multivariable logistic regression analysis, children with more rostral neurological lesion levels had higher odds of sleep disordered breathing (OR for thoracic, mid-lumbar, and low-lumbar: 7.34, 3.70, 4.04, respectively, compared to sacral level, P = .043).

Conclusion: Over 40% of a sample of children with myelomeningocele, who underwent screening polysomnography, had significant sleep disordered breathing. Routine screening polysomnography may be indicated in this population.
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http://dx.doi.org/10.1093/neuros/nyaa507DOI Listing
March 2021

Shunt failure clusters: an analysis of multiple, frequent shunt failures.

J Neurosurg Pediatr 2020 Dec 18:1-7. Epub 2020 Dec 18.

Objective: Repeated failure of ventriculoperitoneal shunts (VPSs) is a problem familiar to pediatric neurosurgeons and patients. While there have been many studies to determine what factors are associated with the first shunt failure, studies of subsequent failures are much less common. The purpose of this study was to identify the prevalence and associated risk factors of clustered shunt failures (defined as 3 or more VPS operations within 3 months).

Methods: The authors reviewed prospectively collected records from all patients who underwent VPS surgery from 2008 to 2017 at their institution and included only those children who had received all of their hydrocephalus care at that institution. Demographics, etiology of hydrocephalus, history of endoscopic third ventriculostomy or temporizing procedure, initial valve type, age at shunt placement, and other factors were analyzed. Logistic regression was used to test for the association of each variable with a history of shunt failure cluster.

Results: Of the 465 included children, 28 (6.0%) had experienced at least one cluster of shunt failures. Among time-independent variables, etiology of hydrocephalus (OR 0.27 for non-intraventricular hemorrhage [IVH], nonmyelomeningocele, nonaqueductal stenosis etiology vs IVH, 95% CI 0.11-0.65; p = 0.003), younger gestational age at birth (OR 0.91, 95% CI 0.85-0.97; p = 0.003), history of a temporizing procedure (OR 2.77, 95% CI 1.12-6.85; p = 0.028), and smaller head circumference at time of initial shunt placement (OR 0.91, 95% CI 0.84-0.99; p = 0.044) showed significant association with shunt failure cluster on univariate analysis. None of these variables maintained significance in a multivariate model. Among children with a history of a shunt failure cluster, 21 (75%) had a shunt infection either prior to or during the shunt failure cluster. A comparison of the infecting organism between these children and 62 children with a history of infection but without a shunt failure cluster showed an association of cluster with gram-negative rod species.

Conclusions: Six percent of children in this institutional sample had at least one shunt failure cluster. These children accounted for 30% of the total shunt revisions in the sample. Shunt infection is an important factor associated with shunt failure cluster. Children with a history of prematurity and IVH may have a higher risk for failure cluster.
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http://dx.doi.org/10.3171/2020.7.PEDS20199DOI Listing
December 2020

Neurosurgery guidelines for the care of people with spina bifida.

J Pediatr Rehabil Med 2020 ;13(4):467-477

Department of Neurosurgery, Alabama Children's Hospital and University of Alabama at Birmingham, Birmingham, AL, USA.

Myelomeningocele (MMC) arises from an early neural developmental anomaly and results in a variety of structural abnormalities and associated functional neurologic deficits. As such, neurologic issues are central to virtually all clinical problems. Neurosurgical intervention strives to correct or improve these defects and prevent secondary complications. These interventions include closure of the open myelomeningocele and management (across the life span) of hydrocephalus, the Chiari II malformation (C2M) and tethered spinal cord (TSC). The development of pre-natal closure techniques and reports of improved outcome with in-utero closure (IUMC) have revolutionized the neurosurgical approach to myelomeningocele. Controversies remain surrounding patient selection, maternal risks, technique of IUMC (endoscopic vs. open) and long-term outcomes. However, real gains include reduced rates of hydrocephalus, modestly improved motor capabilities and reduction in C2M morbidity. For many decades, the cornerstone of treatment of hydrocephalus for many decades has been the placement and support of ventricular shunts. Endoscopic third ventriculostomy (ETV) with or without choroid plexus coagulation (ETV/CPC) is an appealing alternate strategy that avoids the morbidity and complications associated with shunts. The exact criteria for ETV-CPC candidacy and best metrics for outcome analysis remain active areas of debate and controversy. Similarly, neurosurgical management C2M, has centered upon the indications and clinical thresholds for performing posterior fossa surgical decompression. Tethered spinal cord management incorporates the diagnosis and surgical management of adhesions formed at the initial closure site, the consequent longitudinal traction related stress on the cord and the resulting neurologic signs and symptoms.
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http://dx.doi.org/10.3233/PRM-200782DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7838965PMC
January 2020

Post-Traumatic Stress Symptoms in Caregivers and Children with Hydrocephalus.

World Neurosurg 2021 Apr 9;148:e66-e73. Epub 2020 Dec 9.

Department of Neurosurgery, Division of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama, USA.

Objective: Hydrocephalus is a disorder of cerebrospinal fluid dynamics, traditionally treated by placement of a ventricular shunt. Shunts are effective but imperfect as they fail in an unpredictable pattern, and the patient's well-being is dependent on adequate shunt function. The omnipresent threat of shunt failure along with the potential need for invasive investigations can be stressful for patients and caregivers. Our objective was to measure post-traumatic stress symptoms (PTSS) in children with hydrocephalus and their caregivers.

Methods: A cross-sectional analysis of children with hydrocephalus and their caregivers was conducted. Caregivers completed a measure of their own PTSS (the Post-Traumatic Stress Disorders Checklist for the Diagnostic and Statistical Manual of Mental Disorders-V) and resilience (the Connor Davidson Resilience Scale). Pediatric patients rated their own PTSS and resilience using the Acute Stress Checklist for Kids and Connor Davidson Resilience Scale.

Results: Ninety-one caregivers completed the Post-Traumatic Stress Disorders Checklist for the Diagnostic and Statistical Manual of Mental Disorders-V. Mean score was 17.0 (standard deviation 15.7; median 13.0). Fourteen percent scored above 33, the threshold suggestive of a preliminary diagnosis of post-traumatic stress disorder. There was a statistically significant association between caregiver post-traumatic stress and marital status, child's race, and caregiver education. More than half (52%) of caregivers reported their child's hydrocephalus as the most significant source of their PTSS. Children did not have markedly elevated levels of PTSS. Forty-one percent of caregivers and 60% of children scored in the lowest resilience quartile compared with the general population.

Conclusions: Results from this study suggest that post-traumatic stress affects caregivers with hydrocephalus, yet levels of resilience for caregivers and pediatric patients are low.
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http://dx.doi.org/10.1016/j.wneu.2020.12.008DOI Listing
April 2021

Anxiety, depression, fatigue, and headache burden in the pediatric hydrocephalus population.

J Neurosurg Pediatr 2020 Jul 24:1-7. Epub 2020 Jul 24.

1Department of Neurosurgery, Division of Pediatrics; Departments of.

Objective: Childhood hydrocephalus is a common chronic medical condition. However, little is known about the burden of headache and psychological comorbidities in children living with hydrocephalus. The purpose of this study was to determine the prevalence and severity of these conditions among the pediatric hydrocephalus population.

Methods: During routine neurosurgery clinic visits from July 2017 to February 2018, the authors administered four surveys to children ages 7 years and older: Pediatric Migraine Disability Assessment (PedMIDAS), Patient-Reported Outcomes Measurement Information System (PROMIS) Anxiety, PROMIS Depression, and PROMIS Fatigue. The PedMIDAS is an assessment of headache disability in pediatric and adolescent patients. The PROMIS measures are pediatric self-reported instruments to assess social and emotional health. PROMIS measures utilize T-scores (mean 50, SD 10) to compare anxiety, depression, and fatigue in specific populations to those in the US general population. Clinical and demographic data were collected from the medical record (hydrocephalus etiology, shunt infection, race, etc.) and tested for associations with survey measure scores.

Results: Forty children completed the PedMIDAS. Ten percent of them were in the severe headache range, 5% were in the moderate range, and 5% were in the mild range. There was a statistically significant association between undergoing a cluster of shunt operations and headache burden (p = 0.003).Forty children completed all three PROMIS measures. The mean anxiety score was 45.8 (SD 11.7), and 2.5% of children scored in the severe anxiety range, 17.5% in the moderate range, and 20% in the mild range. The mean depression score was 42.7 (SD 10.0), with 2.5% of children scoring in the severe depression range, 5% in the moderate range, and 12.5% in the mild range. The mean fatigue score was 45.1 (SD 16.4), with 15% percent of children scoring in the severe fatigue range, 10% in the moderate range, and 7.5% in the mild range. There were no statistically significant associations between child anxiety, depression, or fatigue and clinical or demographic variables.

Conclusions: Children with hydrocephalus have an average burden of headache, anxiety, depression, and fatigue as compared to the general population overall. Having a cluster of shunt operations correlates with a higher headache burden, but no clinical or demographic variable is associated with anxiety, depression, or fatigue.
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http://dx.doi.org/10.3171/2020.4.PEDS19697DOI Listing
July 2020

Hydrocephalus-related quality of life as assessed by children and their caregivers.

J Neurosurg Pediatr 2020 Jul 10:1-11. Epub 2020 Jul 10.

1Department of Neurosurgery, Division of Pediatric Neurosurgery.

Objective: Hydrocephalus is a chronic medical condition that has a significant impact on children and their caregivers. The objective of this study was to measure the quality of life (QOL) of children with hydrocephalus, as assessed by both caregivers and patients.

Methods: Pediatric patients with hydrocephalus and their caregivers were enrolled during routine neurosurgery clinic visits. The Hydrocephalus Outcomes Questionnaire (HOQ), a report of hydrocephalus-related QOL, was administered to both children with hydrocephalus (self-report) and their caregivers (proxy report about the child). Patients with hydrocephalus also completed measures of anxiety, depression, fatigue, traumatic stress, and headache. Caregivers completed a proxy report of child traumatic stress and a measure of caregiver burden. Demographic information was collected from administration of the Psychosocial Assessment Tool (version 2.0) and from the medical record. Child and caregiver HOQ scores were analyzed and correlated with clinical, demographic, and psychological variables.

Results: The mean overall HOQ score (parent assessment of child QOL) was 0.68. HOQ Physical Health, Social-Emotional Health, and Cognitive Health subscore averages were 0.69, 0.73, and 0.54, respectively. The mean overall child self-assessment (cHOQ) score was 0.77, with cHOQ Physical Health, Social-Emotional Health, and Cognitive Health subscore means of 0.84, 0.79, and 0.66, respectively. Thirty-nine dyads were analyzed, in which both a child with hydrocephalus and his or her caregiver completed the cHOQ and HOQ. There was a positive correlation between parent and child scores (p < 0.004 for all subscores). Child scores were consistently higher than parent scores. Variables that showed association with caregiver-assessed QOL in at least one domain included child age, etiology of hydrocephalus, and history of endoscopic third ventriculostomy. There was a significant negative relationship (rho -0.48 to -0.60) between child-reported cHOQ score and child-reported measures of posttraumatic stress, anxiety, depression, and fatigue. There was a similar significant relationship between caregiver report of child's QOL (HOQ) and caregiver assessment of the child's posttraumatic stress symptoms as well as their assessment of burden of care (rho = -0.59 and rho = -0.51, respectively). No relationship between parent-reported HOQ and child-reported psychosocial factors was significant. No clinical or demographic variables were associated with child self-assessed cHOQ.

Conclusions: Pediatric patients with hydrocephalus consistently rate their own QOL higher than their caregivers do. Psychological factors such as anxiety and posttraumatic stress may be associated with lower QOL. These findings warrant further exploration.
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http://dx.doi.org/10.3171/2020.4.PEDS19660DOI Listing
July 2020

The impact of imposed delay in elective pediatric neurosurgery: an informed hierarchy of need in the time of mass casualty crisis.

Childs Nerv Syst 2020 07 20;36(7):1347-1355. Epub 2020 May 20.

Department of Neurosurgery, University of Alabama at Birmingham, Children's of Alabama, Birmingham, AL, USA.

SARS-CoV-2 COVID-19, coronavirus, has created unique challenges for the medical community after national guidelines called for the cancellation of all elective surgery. While there are clear cases of elective surgery (benign cranial cosmetic defect) and emergency surgery (hemorrhage, fracture, trauma, etc.), there is an unchartered middle ground in pediatric neurosurgery. Children, unlike adults, have dynamic anatomy and are still developing neural networks. Delaying seemingly elective surgery can affect a child's already vulnerable health state by further impacting their neurocognitive development, neurologic functioning, and potential long-term health states. The purpose of this paper is to demonstrate that "elective" pediatric neurosurgery should be risk-stratified, and multi-institutional informed guidelines established.
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http://dx.doi.org/10.1007/s00381-020-04671-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7239607PMC
July 2020

Pilot Study of Neurodevelopmental Impact of Early Epilepsy Surgery in Tuberous Sclerosis Complex.

Pediatr Neurol 2020 08 14;109:39-46. Epub 2020 Apr 14.

Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama. Electronic address:

Background: To determine if early epilepsy surgery mitigates detrimental effects of refractory epilepsy on development, we investigated surgical and neurodevelopmental outcomes in children with tuberous sclerosis complex who underwent surgery before age two years.

Methods: Prospective multicenter observational study of 160 children with tuberous sclerosis complex. Surgical outcome was determined for the seizure type targeted by surgery. We obtained Vineland Adaptive Behavior Scales, Second Edition (Vineland-II); Mullen Scales of Early Learning; and Preschool Language Scales, Fifth Edition, at age three, six, nine, 12, 18, 24, and 36 months. Surgical cases were compared with children without seizures, with controlled seizures, and with medically refractory seizures.

Results: Nineteen children underwent surgery (median age 17 months, range 3.7 to 21.3), and mean follow-up was 22.8 months (range 12 to 48). Surgical outcomes were favorable in 12 (63%, Engel I-II) and poor in seven (37%, Engel III-IV). Nine (47%) had new or ongoing seizures distinct from those surgically targeted. All children with seizures demonstrated longitudinal decline or attenuated gains in neurodevelopment, the surgical group scoring the lowest. Favorable surgical outcome was associated with increased Mullen Scales of Early Learning receptive and expressive language subscores compared with the medically refractory seizure group. A nonsignificant but consistent pattern of improvement with surgery was seen in all tested domains.

Conclusions: These pilot data show neurodevelopmental gains in some domains following epilepsy surgery. A properly powered, prospective multicenter observational study of early epilepsy surgery is needed, using both surgical and developmental outcome metrics.
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http://dx.doi.org/10.1016/j.pediatrneurol.2020.04.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7387194PMC
August 2020

Relative contribution of individual versus combined functional imaging studies in predicting seizure freedom in pediatric epilepsy surgery: an area under the curve analysis.

Neurosurg Focus 2020 04;48(4):E13

6Division of Pediatric Neurosurgery, Department of Neurosurgery, The University of Alabama at Birmingham School of Medicine, Birmingham, Alabama; and.

Objective: The goal of this study was to evaluate the predictive value and relative contribution of noninvasive presurgical functional imaging modalities based on the authors' institutional experience in pursuing seizure-free surgical outcomes in children with medically refractory epilepsy.

Methods: This was a retrospective, single-institution, observational cohort study of pediatric patients who underwent evaluation and surgical treatment for medically refractory partial epilepsy between December 2003 and June 2016. During this interval, 108 children with medically refractory partial epilepsy underwent evaluation for localization and resective epilepsy surgery. Different noninvasive functional imaging modalities, including ictal SPECT, FDG-PET, and magnetoencephalography-magnetic source imaging, were utilized to augment a standardized paradigm (electroencephalography/semiology, MRI, and neuropsychology findings) for localization. Outcomes were evaluated at a minimum of 2 years (mean 7.5 years) utilizing area under the receiver operating characteristic curve analysis. Localizing modalities and other clinical covariates were examined in relation to long-term surgical outcomes.

Results: There was variation in the contribution of each test, and no single presurgical workup modality could singularly and reliably predict a seizure-free outcome. However, concordance of presurgical modalities yielded a high predictive value. No difference in long-term outcomes between inconclusive (normal or diffusely abnormal) and abnormal focal MRI results were found. Long-term survival analyses revealed a statistically significant association between seizure freedom and patients with focal ictal EEG, early surgical intervention, and no history of generalized convulsions.

Conclusions: Comprehensive preoperative evaluation utilizing multiple noninvasive functional imaging modalities is not redundant and can improve pediatric epilepsy surgical outcomes.
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http://dx.doi.org/10.3171/2020.1.FOCUS19974DOI Listing
April 2020

Health care transition in pediatric neurosurgery: a consensus statement from the American Society of Pediatric Neurosurgeons.

J Neurosurg Pediatr 2020 Feb 14:1-9. Epub 2020 Feb 14.

9Department of Surgery, Division of Neurosurgery, Connecticut Children's Medical Center, Hartford, Connecticut.

Objective: The number of children with complex medical conditions surviving to adulthood is increasing. A planned transition to adult care systems is essential to the health maintenance of these patients. Guidance has been established for the general health care transition (HCT) from adolescence to adulthood. No formal assessment of the performance of pediatric neurosurgeons in HCT has been previously performed. No "best practice" for this process in pediatric neurosurgery currently exists. The authors pursued two goals in this paper: 1) define the current state of HCT in pediatric neurosurgery through a survey of the membership of the American Society of Pediatric Neurosurgeons (ASPN) on current methods of HCT, and 2) develop leadership-endorsed best-practice guidelines for HCT from pediatric to adult neurosurgical health care.

Methods: Completion of the Current Assessment of Health Care Transition Activities survey was requested of 178 North American pediatric neurosurgeons by using a web-based questionnaire to capture HCT practices of the ASPN membership. The authors concurrently conducted a PubMed/MEDLINE-based literature review of HCT for young adults with special health care needs, surgical conditions, and/or neurological conditions for the period from 1990 to 2018. Selected articles were assembled and reviewed by subject matter experts and members of the ASPN Quality, Safety, and Advocacy Committee. Best-practice recommendations were developed and subjected to peer review by external expert groups.

Results: Seventy-six responses to the survey (43%) were received, and 62 respondents (82%) answered all 12 questions. Scores of 1 (lowest possible score) were recorded by nearly 60% of respondents on transition policy, by almost 70% on transition tracking, by 85% on transition readiness, by at least 40% on transition planning as well as transfer of care, and by 53% on transition completion. Average responses on all core elements were < 2 on the established 4-point scale. Seven best-practice recommendations were developed and endorsed by the ASPN leadership.

Conclusions: The majority of pediatric neurosurgeons have transition practices that are poor, do not meet the needs of patients and families, and should be improved. A structured approach to transition, local engagement with adult neurosurgical providers, and national partnerships between pediatric and adult neurosurgery organizations are suggested to address current gaps in HCT for patients served by pediatric neurosurgeons.
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http://dx.doi.org/10.3171/2019.12.PEDS19524DOI Listing
February 2020

Functional outcomes at 2 years of age following treatment for posthemorrhagic hydrocephalus of prematurity: what do we know at the time of consult?

J Neurosurg Pediatr 2020 Feb 14:1-9. Epub 2020 Feb 14.

1Department of Neurosurgery, Division of Pediatric Neurosurgery.

Objective: Posthemorrhagic hydrocephalus of prematurity remains a significant problem in preterm infants. In the literature, there is a scarcity of data on the early disease process, when neurosurgeons are typically consulted for recommendations on treatment. Here, the authors sought to evaluate functional outcomes in premature infants at 2 years of age following treatment for posthemorrhagic hydrocephalus. Their goal was to determine the relationship between factors identifiable at the time of the initial neurosurgical consult and outcomes of patients when they are 2 years of age.

Methods: The authors performed a retrospective chart review of premature infants treated for intraventricular hemorrhage (IVH) of prematurity (grade III and IV) between 2003 and 2014. Information from three time points (birth, first neurosurgical consult, and 2 years of age) was collected on each patient. Logistic regression analysis was performed to determine the association between variables known at the time of the first neurosurgical consult and each of the outcome variables.

Results: One hundred thirty patients were selected for analysis. At 2 years of age, 16% of the patients had died, 88% had cerebral palsy/developmental delay (CP), 48% were nonverbal, 55% were nonambulatory, 33% had epilepsy, and 41% had visual impairment. In the logistic regression analysis, IVH grade was an independent predictor of CP (p = 0.004), which had an estimated probability of occurrence of 74% in grade III and 96% in grade IV. Sepsis at or before the time of consult was an independent predictor of visual impairment (p = 0.024), which had an estimated probability of 58%. IVH grade was an independent predictor of epilepsy (p = 0.026), which had an estimated probability of 18% in grade III and 43% in grade IV. The IVH grade was also an independent predictor of verbal function (p = 0.007), which had an estimated probability of 68% in grade III versus 41% in grade IV. A higher weeks gestational age (WGA) at birth was an independent predictor of the ability to ambulate (p = 0.0014), which had an estimated probability of 15% at 22 WGA and up to 98% at 36 WGA. The need for oscillating ventilation at consult was an independent predictor of death before 2 years of age (p = 0.001), which had an estimated probability of 42% in patients needing oscillating ventilation versus 13% in those who did not.

Conclusions: IVH grade was consistently an independent predictor of functional outcomes at 2 years. Gestational age at birth, sepsis, and the need for oscillating ventilation may also predict worse functional outcomes.
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http://dx.doi.org/10.3171/2019.12.PEDS19381DOI Listing
February 2020

Incidence of delayed intracranial hypertension in children with isolated sagittal synostosis following open calvarial vault reconstruction.

Childs Nerv Syst 2020 03 5;36(3):545-550. Epub 2019 Dec 5.

Department of Neurosurgery, University of Alabama at Birmingham School of Medicine, 1057 Faculty Office Tower, 510 20th Street South, Birmingham, AL, 35294-3410, USA.

Purpose: Delayed intracranial hypertension (DIH) occurs most frequently in children with syndromic or multi-suture synostosis after surgical correction. The rarity of DIH in children with isolated non-syndromic sagittal synostosis (ISS) warrants follow-up evaluation by large craniofacial centers until skeletal maturity. This study reports the incidence of DIH in children following open repair for ISS by our center's craniofacial team.

Methods: A single-center retrospective study of patients who underwent open calvarial vault remodeling (CVR) for ISS at our institution between November 2000 and November 2012 was performed. Syndromic and multi-suture synostosis patients were excluded. Demographic and follow-up data were extracted from the medical record for analysis until July 2017.

Results: One hundred five patients with ISS were identified who had undergone CVR in the aforementioned timeframe. Average age at initial surgery was 11.7 ± 15.32 months. Mean follow-up in our craniofacial clinic was 4.94 ± 3.53 years, with 69 patients (65.7%) having follow-up in craniofacial clinic ≥ 3 years and 74 (70.5%) having follow-up ≥ 3 years in any clinic at our institution. Four patients (3.8%) had intracranial pressure (ICP) monitors placed for symptoms concerning for DIH, one of which (0.95%) had confirmed DIH and underwent a second surgical procedure at 7.4 years of age. The patient presented late initially, having his first operation at 1.56 years of age.

Conclusion: One patient out of 105 (0.95%) developed DIH, confirmed by ICP monitoring, and required reoperation. The occurrence of DIH, albeit rare, remains an important topic to include in parental discussions and mandates long-term follow-up in this population.
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http://dx.doi.org/10.1007/s00381-019-04406-7DOI Listing
March 2020

Letter to the Editor. Fetal closure of myelomeningocele.

J Neurosurg Pediatr 2019 Nov 29:1-3. Epub 2019 Nov 29.

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http://dx.doi.org/10.3171/2019.8.PEDS19498DOI Listing
November 2019

Concepts in the neurosurgical care of patients with spinal neural tube defects: An embryologic approach.

Birth Defects Res 2019 11 2;111(19):1564-1576. Epub 2019 Oct 2.

University of Wisconsin School of Medicine, Madison, Wisconsin.

Background: The neural tube defects (NTDs) are a heterogeneous group of structural birth defects that arise from a complex array of multiple genetic and environmental factors and adversely affect the structure and function of the brain and spinal cord. Spinal NTDs are clinically more common than cranial NTDs. There remains a significant gap in linking the multiple NTD phenotypes to current genomic understanding.

Methods: This article summarizes the neurosurgical clinical approach to spinal NTDs by correlating each step of embryonic development of the human nervous system with key management concepts for defects that arise at that step.

Results: The NTDs are broadly classified as open or closed. Open defects include myelomeningocele (MMC), encephalocele, and anencephaly. Closed defects are also known as occult spinal dysraphism and are characterized by intact skin over the spinal defect. They are more common and often cause neurologic decline from tethered cord syndrome. Failure of primary neurulation gives rise to open myelomeningocele (MMC). Surgical closure of an open MMC focuses on realigning the tissue layers that failed to separate during neurulation. In utero closure is a promising recent technique. Chronic neurosurgical management largely focuses treating hydrocephalus. The Chiari II malformation is uniformly present in MMC patients and may cause brainstem dysfunction. Tethered spinal cord may progressively impair normal neurologic function but typically responds well to surgical untethering.

Conclusions: Surgical closure of MMC centers on approximated realignment of embryologically disordered neural tissue. Clinical surgical management decisions in the spinal NTDs remains challenging but standardized principles have emerged.
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http://dx.doi.org/10.1002/bdr2.1588DOI Listing
November 2019

Development of an evidence-based individualized transition plan for spina bifida.

Neurosurg Focus 2019 10;47(4):E17

1Department of Neurosurgery and.

Objective: In spina bifida (SB), transition of care from the pediatric to adult healthcare settings remains an opportunity for improvement. Transition of care is necessarily multidimensional and focuses on increasing independence, autonomy, and personal responsibility for health-related tasks. While prior research has demonstrated that effective transition can improve health outcomes and quality of life while reducing healthcare utilization, little is known about the most advantageous transition program components/design. The individualized transition plan (ITP) was developed to optimize the readiness of the adolescent with SB for adult healthcare. The ITP is a set of clearly articulated, mutually developed goals that arise from best available data on successful transition and are individualized to meet the individual challenges, needs, and attributes of each patient and family.

Methods: Prospectively completed ITPs were retrospectively reviewed from June 2018 to May 2019. Demographic and disease characteristics were collected, and specific goals were reviewed and categorized.

Results: Thirty-two patients with an ITP were included. The cohort was 50% male and had a mean age of 16.4 years. For goal 1 (maximize education), the most common goal was to complete a career interest survey (44%), followed by researching application/admission requirements for programs of interest (25%), shadowing in and/or visiting a workplace (16%), and improving high school performance (16%). For goal 2 (bowel management), most patients (59%) had a working bowel program with few or no bowel accidents. Eight patients (25%) were having more than the desired number of bowel accidents and received formal consultation with a gastroenterologist. Five patients (16%) needed only minor adjustments to their bowel management regimen. Goal 3 (SB program coordinator goal) focused on documenting medical and/or surgical history for the majority of patients (66%). Other goals aimed to increase patient communication in healthcare settings or utilize available community resources.

Conclusions: The authors developed an evidence-based ITP that focuses around 5 goals: maximizing education, bowel continence, and goals set by the SB clinic coordinator, parent/caregiver, and patient. Although developed for the authors' SB clinic, the ITP concept is applicable to transition of care in any chronic childhood illness.
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http://dx.doi.org/10.3171/2019.7.FOCUS19425DOI Listing
October 2019

Introduction. Advancing the care of children with spina bifida, prenatally and postnatally.

Neurosurg Focus 2019 10;47(4):E1

5Department of Neurosurgery, VIMS and Park Clinic, Kolkata, India.

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http://dx.doi.org/10.3171/2019.8.FOCUS19666DOI Listing
October 2019

Care management and contemporary challenges in spina bifida: a practice preference survey of the American Society of Pediatric Neurosurgeons.

J Neurosurg Pediatr 2019 Aug 30:1-10. Epub 2019 Aug 30.

1Department of Neurosurgery, University of Alabama at Birmingham.

Objective: Neurosurgical management preferences related to myelomeningocele (MMC) care demonstrate significant variability. The authors sought to evaluate variability in practice patterns across a group of senior pediatric neurosurgeons. The purpose of this study was to identify the extent of variability and of consensus with regard to neurosurgical management of MMC and associated hydrocephalus, Chiari II malformation, and tethered spinal cord.

Methods: A 43-question survey was distributed electronically to the members of the American Society of Pediatric Neurosurgeons (ASPN). The survey covered domains such as clinic case volume, newborn management, hydrocephalus management, transition to adulthood, clinical indications for shunt revision, Chiari II malformation decompression (C2MD), and tethered cord release (TCR). Ninety responses were received from 200 active ASPN members, for an overall response rate of 45%.

Results: The majority (58%) of respondents closed 5-15 new cases of open MMC per year. Nearly all (98%) respondents perform back closure within 48 hours of birth, with the majority imbricating the placode and striving for a 3- to 4-layer closure. The most consistent indications for surgical intervention in early hydrocephalus were CSF leak from the back (92%), progressive ventricular enlargement (89%), and brainstem symptoms, including apnea/bradycardia (81%), stridor (81%), and dysphagia (81%). Eighty percent of respondents indicated that spina bifida care is delivered through multidisciplinary clinics, with neurosurgery, orthopedic surgery, urology, physical therapy, and social work as the most common disciplines included. One-third of clinics see both pediatric and adult patients, one-third offer a formal transition program to adult care, and one-third have no transition program. The vast majority of respondents offer prenatal counseling (95%), referral for in utero closure (66%), and endoscopic third ventriculostomy/choroid plexus cauterization (72%). Respondents were more willing to perform shunt revision for symptoms alone than for image changes alone. An asymptomatic broken shunt without ventricular enlargement produced responses evenly divided between observation, intervention, and further investigation. Operative shunt exploration was always performed before C2MD by 56% of respondents and performed sometimes by 40% of respondents. Symptoms of brainstem dysfunction were the strongest clinical triggers reported for C2MD, while declines in urinary continence, leg strength or sensation, or ambulation were the most consistent thresholds for TCR.

Conclusions: Significant disparities exist surrounding key areas of decision making regarding treatment for patients with MMC, though there are central areas of agreement among ASPN members. Additionally, there is significant variation in the clinical management of chronic hydrocephalus, C2MD, and TCR, underscoring the need for further research into these specific areas.
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http://dx.doi.org/10.3171/2019.5.PEDS18738DOI Listing
August 2019

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on Closure of Myelomeningocele Within 48 Hours to Decrease Infection Risk.

Neurosurgery 2019 09;85(3):E412-E413

Goryeb Children's Hospital, Morristown, NJ; Rutgers Department of Neurological Surgery, Newark, New Jersey.

Background: Appropriate timing for closure of myelomeningocele (MM) varies in the literature. Older studies present 48 h as the timeframe after which infection complication rates rise.

Objective: The objective of this guideline is to determine if closing the MM within 48 h decreases the risk of wound infection or ventriculitis.

Methods: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review. Full text articles were then reviewed and when appropriate, included in the evidentiary table. The class of evidence was evaluated, discussed, and assigned to each study that met inclusion criteria.

Results: A total of 148 abstracts were identified and reviewed. A total of 31 articles were selected for full text analysis. Only 4 of these studies met inclusion criteria.

Conclusion: There is insufficient evidence that operating within 48 h decreases risk of wound infection or ventriculitis in 1 Class III study. There is 1 Class III study that provides evidence of global increase in postoperative infection after 48 h, but is not specific to wound infection or ventriculitis. There is 1 Class III study that provides evidence if surgery is going to be delayed greater than 48 h, antibiotics should be given.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-4.
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http://dx.doi.org/10.1093/neuros/nyz264DOI Listing
September 2019

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Incidence of Shunt-Dependent Hydrocephalus in Infants With Myelomeningocele After Prenatal Versus Postnatal Repair.

Neurosurgery 2019 09;85(3):E405-E408

Goryeb Children's Hospital, Morristown, NJ; Rutgers Department of Neurological Surgery, Newark, New Jersey.

Background: Myelomeningocele (MM) is a condition that is responsible for considerable morbidity in the pediatric population. A significant proportion of the morbidity related to MM is attributable to hydrocephalus and the surgical management thereof. Postnatal repair remains the most common form of treatment; however, increased rates of prenatal diagnosis, advances in fetal surgery, and a hypothesis that neural injury continues in utero until the MM defect is repaired have led to the development and evaluation of prenatal surgery as a means to improve outcomes in afflicted infants.

Objective: The objective of this guideline is to systematically evaluate the literature to determine whether there is a difference in the proportion of patients who develop shunt-dependent hydrocephalus in infants who underwent prenatal MM repair compared to infants who had postnatal repair.

Methods: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review. Full-text articles were then reviewed, and when appropriate, included as evidence.

Results: A total of 87 abstracts were identified and reviewed by 3 independent reviewers. Thirty-nine full-text articles were selected for analysis. Three studies met selection criteria and were included in the evidence table.

Conclusion: Class I evidence from 1 study and class III evidence from 2 studies suggest that, in comparison to postnatal repair, prenatal surgery for MM reduces the risk of developing shunt-dependent hydrocephalus. Therefore, prenatal repair of MM is recommended for those fetuses who meet specific criteria for prenatal surgery to reduce the risk of developing shunt-dependent hydrocephalus (level I). Differences between prenatal and postnatal repair with respect to the requirement for permanent cerebrospinal fluid diversion should be considered alongside other relevant maternal and fetal factors when deciding upon a preferred method of MM closure. The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-2.
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http://dx.doi.org/10.1093/neuros/nyz262DOI Listing
September 2019

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Pediatric Myelomeningocele: Executive Summary.

Neurosurgery 2019 09;85(3):299-301

Department of Neurosurgery, Mercer University Medical School, Macon, Georgia.

Background: The incidence of spina bifida (SB) in the developing world is higher than in the United States because of malnutrition and folic acid deficiency during pregnancy. Advances in technology have made prenatal repair of myelomeningocele (MM) possible.

Objective: The objective of the guidelines are, (1) To create clinical recommendations for best practices, based on a systematic review and analysis of available literature, (2) to obtain multi-disciplinary endorsement of these guidelines from relevant organizations, and (3) to disseminate the educational content to physicians to improve the care of infants with MM.

Methods: The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review.

Results: Guidelines authors aimed to systematically review the literature and make evidence based recommendations about the timing of closure after birth, hydrocephalus, the impact of prenatal closure, and the effect of prenatal closure on ambulation ability and tethered spinal cord. Evidence concerning persistent ventriculomegaly and cognitive impairment was also evaluated. Hundreds of abstracts were identified and reviewed for each of the 5 topics. A total of 14 studies met stringent inclusion criteria.

Conclusion: Based on a comprehensive systematic review, a total of 5 clinical practice recommendations were developed, with 1 Level I, 2 Level II and 2 Level III recommendations.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-1.
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http://dx.doi.org/10.1093/neuros/nyz261DOI Listing
September 2019

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Incidence of Tethered Cord Syndrome in Infants With Myelomeningocele With Prenatal Versus Postnatal Repair.

Neurosurgery 2019 09;85(3):E417-E419

Division of Pediatric Neurosurgery, British Columbia Children's Hospital, University of British Columbia, Vancouver, Canada.

Background: The incidence of spina bifida (SB) is higher in the developing world as compared to the United States because of folic acid deficiency during pregnancy. Advances in technology have made prenatal repair of myelomeningocele (MM) possible.

Objective: The objective of this guideline was to determine if there is a difference in the rate of development of tethered cord syndrome (TCS) in infants who had prenatal closure compared to infants who had MM repair after birth.

Methods: The Guidelines Task Force developed search terms and strategies to search PubMed and Embase for the relevant literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used. Full text articles were reviewed and, when appropriate, included as evidence.

Results: A total of 261 abstracts were reviewed. Fifty-four full-text articles were selected for further analysis. Three studies met inclusion criteria.

Conclusion: There was Class II evidence from 1 study and Class III evidence from another 2 studies demonstrating that TCS develops in infants with prenatal MM closure at an equal or higher rate than with postnatal closure. There was an increased risk of development of inclusion cysts in infants who underwent in utero closure. Continued surveillance for TCS and/or the development of inclusion cysts in children with prenatal and postnatal closure of MM is indicated (Level II). Differences between prenatal and postnatal repair with respect to the development of TCS and/or inclusion cysts should be considered alongside other relevant maternal and fetal outcomes when deciding upon a preferred method for MM closure.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-6.
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http://dx.doi.org/10.1093/neuros/nyz266DOI Listing
September 2019

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guideline on the Management of Patients With Myelomeningocele: Whether Persistent Ventriculomegaly Adversely Impacts Neurocognitive Development.

Neurosurgery 2019 09;85(3):E414-E416

Goryeb Children's Hospital, Morristown, New Jersey.

Background: Myelomeningocele (MM) is the most common congenital anomaly to affect the nervous system and affects 1500-2000 newborn infants per year in the United States. It is accompanied by symptomatic hydrocephalus in approximately 70%-80% of patients. Different treatment strategies for hydrocephalus characteristically result in different effects on the size of the ventricles.

Objective: The objective of this systematic review was to determine whether persistent ventricular enlargement adversely impacts neurocognitive development in patients with MM.

Methods: The PubMed National Library of Medicine Medline database and Embase were queried using MeSH headings and keywords relevant to neurocognitive or intellectual development and ventricular size or morphology. Abstracts were reviewed by the authors to identify which studies met strict inclusion criteria. An evidence table was constructed that summarized the included studies and reflected the quality of evidence (Classes I-III) that each represented. A recommendation was made that is based on the quality of the evidence.

Results: An initial abstract review utilizing strict inclusion/exclusion criteria yielded 48 studies, 9 of which underwent full-text review. There is limited and conflicting Class III evidence from 2 studies.

Conclusion: Currently, there is insufficient data to conclude that ventricular size and morphology impact neurocognitive development.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-5.
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http://dx.doi.org/10.1093/neuros/nyz265DOI Listing
September 2019

Commentary: Open Resection versus Laser Interstitial Thermal Therapy for the Treatment of Pediatric Insular Epilepsy.

Authors:
Jeffrey P Blount

Neurosurgery 2019 10;85(4):E737-E738

Department of Neurosurgery, School of Medicine, University of Alabama at Birmingham, and Children's of Alabama, Birmingham, Alabama.

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http://dx.doi.org/10.1093/neuros/nyz187DOI Listing
October 2019