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Ian F. Pollack, Sameer Agnihotri and Alberto Broniscer

Brain tumors are the most common solid tumors in children, and, unfortunately, many subtypes continue to have a suboptimal long-term outcome. During the last several years, however, remarkable advances in our understanding of the molecular underpinnings of these tumors have occurred as a result of high-resolution genomic, epigenetic, and transcriptomic profiling, which have provided insights for improved tumor categorization and molecularly directed therapies. While tumors such as medulloblastomas have been historically grouped into standard- and high-risk categories, it is now recognized that these tumors encompass four or more molecular subsets with distinct clinical and molecular characteristics. Likewise, high-grade glioma, which for decades was considered a single high-risk entity, is now known to comprise multiple subsets of tumors that differ in terms of patient age, tumor location, and prognosis. The situation is even more complex for ependymoma, for which at least nine subsets of tumors have been described. Conversely, the majority of pilocytic astrocytomas appear to result from genetic changes that alter a single, therapeutically targetable molecular pathway. Accordingly, the present era is one in which treatment is evolving from the historical standard of radiation and conventional chemotherapy to a more nuanced approach in which these modalities are applied in a risk-adapted framework and molecularly targeted therapies are implemented to augment or, in some cases, replace conventional therapy. Herein, the authors review advances in the categorization and treatment of several of the more common pediatric brain tumors and discuss current and future directions in tumor management that hold significant promise for patients with these challenging tumors.

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Prospective multicenter studies in pediatric hydrocephalus

JNSPG 75th Anniversary Invited Review Article

John R. W. Kestle and Jay Riva-Cambrin

Prospective multicenter clinical research studies in pediatric hydrocephalus are relatively rare. They cover a broad spectrum of hydrocephalus topics, including management of intraventricular hemorrhage in premature infants, shunt techniques and equipment, shunt outcomes, endoscopic treatment of hydrocephalus, and prevention and treatment of infection. The research methodologies include randomized trials, cohort studies, and registry-based studies. This review describes prospective multicenter studies in pediatric hydrocephalus since 1990. Many studies have included all forms of hydrocephalus and used device or procedure failure as the primary outcome. Although such studies have yielded useful findings, they might miss important treatment effects in specific subgroups. As multicenter study networks grow, larger patient numbers will allow studies with more focused entry criteria based on known and evolving prognostic factors. In addition, increased use of patient-centered outcomes such as neurodevelopmental assessment and quality of life should be measured and emphasized in study results. Well-planned multicenter clinical studies can significantly affect the care of children with hydrocephalus and will continue to have an important role in improving care for these children and their families.

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Spinal instrumentation in infants, children, and adolescents: a review

JNSPG 75th Anniversary Invited Review Article

Stephen Mendenhall, Dillon Mobasser, Katherine Relyea and Andrew Jea

OBJECTIVE

The evolution of pediatric spinal instrumentation has progressed in the last 70 years since the popularization of the Harrington rod showing the feasibility of placing spinal instrumentation into the pediatric spine. Although lacking in pediatric-specific spinal instrumentation, when possible, adult instrumentation techniques and tools have been adapted for the pediatric spine. A new generation of pediatric neurosurgeons with interest in complex spine disorder has pushed the field forward, while keeping the special nuances of the growing immature spine in mind. The authors sought to review their own experience with various types of spinal instrumentation in the pediatric spine and document the state of the art for pediatric spine surgery.

METHODS

The authors retrospectively reviewed patients in their practice who underwent complex spine surgery. Patient demographics, operative data, and perioperative complications were recorded. At the same time, the authors surveyed the literature for spinal instrumentation techniques that have been utilized in the pediatric spine. The authors chronicle the past and present of pediatric spinal instrumentation, and speculate about its future.

RESULTS

The medical records of the first 361 patients who underwent 384 procedures involving spinal instrumentation from July 1, 2007, to May 31, 2018, were analyzed. The mean age at surgery was 12 years and 6 months (range 3 months to 21 years and 4 months). The types of spinal instrumentation utilized included occipital screws (94 cases); C1 lateral mass screws (115 cases); C2 pars/translaminar screws (143 cases); subaxial cervical lateral mass screws (95 cases); thoracic and lumbar spine traditional-trajectory and cortical-trajectory pedicle screws (234 cases); thoracic and lumbar sublaminar, subtransverse, and subcostal polyester bands (65 cases); S1 pedicle screws (103 cases); and S2 alar-iliac/iliac screws (56 cases). Complications related to spinal instrumentation included hardware-related skin breakdown (1.8%), infection (1.8%), proximal junctional kyphosis (1.0%), pseudarthroses (1.0%), screw malpositioning (0.5%), CSF leak (0.5%), hardware failure (0.5%), graft migration (0.3%), nerve root injury (0.3%), and vertebral artery injury (0.3%).

CONCLUSIONS

Pediatric neurosurgeons with an interest in complex spine disorders in children should develop a comprehensive armamentarium of safe techniques for placing rigid and nonrigid spinal instrumentation even in the smallest of children, with low complication rates. The authors’ review provides some benchmarks and outcomes for comparison, and furnishes a historical perspective of the past and future of pediatric spine surgery.

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Mireille E. Kelley, Mark A. Espeland, William C. Flood, Alexander K. Powers, Christopher T. Whitlow, Joseph A. Maldjian, Joel D. Stitzel and Jillian E. Urban

OBJECTIVE

Limiting contact in football practice can reduce the number of head impacts a player receives, but further research is needed to inform the modification of optimal drills that mitigate head impact exposure (HIE) while the player develops the skills needed to safely play the game. This study aimed to compare HIE in practice drills among 6 youth football teams and to evaluate the effect of a team on HIE.

METHODS

On-field head impact data were collected from athletes (ages 10–13 years) playing on 6 local youth football teams (teams A–F) during all practices using the Head Impact Telemetry System. Video was recorded and analyzed to verify and assign impacts to a specific drill. Drills were identified as follows: dummy/sled tackling, half install, install, install walk through, multiplayer tackle, Oklahoma, one-on-one, open field tackling, other, passing, position skill work, scrimmage, special teams, tackling drill stations, and technique. HIE was quantified in terms of impacts per player per minute (ppm) and peak linear and rotational head acceleration. Generalized linear models were used to assess differences in head impact magnitude and frequency among drills as well as among teams within the most common drills.

RESULTS

Among 67 athlete-seasons, a total of 14,718 impacts during contact practices were collected and evaluated in this study. Among all 6 teams, the mean linear (p < 0.0001) and rotational (p < 0.0001) acceleration varied significantly among all drills. Open field tackling had significantly (p < 0.001) higher mean linear acceleration than all other drills. Multiplayer tackle had the highest mean impact rate (0.35 ppm). Significant variations in linear acceleration and impact rate were observed among teams within specific drills. Team A had the highest mean linear acceleration in install, one-on-one, and open field tackling and the highest mean impact rate in Oklahoma and position skill work. Although team A spent the greatest proportion of their practice on minimal- or no-player versus player contact drills (27%) compared to other teams, they had the highest median (20.2g) and 95th percentile (56.4g) linear acceleration in practice.

CONCLUSIONS

Full-speed tackling and blocking drills resulted in the highest HIE. Reducing time spent on contact drills relative to minimal or no contact drills may not lower overall HIE. Instead, interventions such as reducing the speed of players engaged in contact, correcting tackling technique, and progressing to contact may reduce HIE more effectively.

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Atilio E. Palma, Robert T. Wicks, Gautam Popli and Daniel E. Couture

Corpus callosotomy has been used as a form of surgical palliation for patients suffering from medically refractory generalized seizures, including drop attacks. Callosotomy has traditionally been described as involving a craniotomy with microdissection. MR-guided laser interstitial thermal therapy (MRg-LITT) has recently been used as a minimally invasive method for performing surgical ablation of epileptogenic foci and corpus callosotomy. The authors present 3 cases in which MRg-LITT was used to perform a corpus callosotomy as part of a staged surgical procedure for a patient with multiple seizure types and in instances when further ablation of residual corpus callosum is necessary after a prior open surgical procedure. To the authors’ knowledge, this is the first case series of corpus callosotomy performed using the MRg-LITT system with a 3.3-year average follow-up. Although MRg-LITT is not expected to replace the traditional corpus callosotomy in all cases, it is a safe, effective, and durable alternative to the traditional open corpus callosotomy, particularly in the setting of a prior craniotomy.

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Nickalus R. Khan, Kenneth Moore, Jaafar Basma, David S. Hersh, Asim F. Choudhri, Brandy Vaughn and Paul Klimo Jr.

OBJECTIVE

An ischemic stroke following an elective craniotomy in a child is perceived to be a rare event. However, to date there are few papers on this topic. The purpose of this study was to investigate the occurrence of stroke following elective intracranial surgery at a children’s hospital.

METHODS

The authors performed a retrospective review of all patients who developed a perioperative stroke following an elective craniotomy from 2010 through 2017. Data were collected using an institutional database that contained demographic, medical, radiological, and outcome variables.

RESULTS

A total of 1591 elective craniotomies were performed at the authors’ institution during the study period. Of these, 28 (1.8%) were followed by a perioperative stroke. Radiographic diagnosis of the infarction occurred at a median of 1.7 days (range 0–9 days) from the time of surgery, and neurological deficits were apparent within 24 hours of surgery in 18 patients (62.5%). Infarcts tended to occur adjacent to tumor resection sites (86% of cases), and in a unilateral (89%), unifocal (93%), and supratentorial (93%) location. Overall, 11 (39.3%) strokes were due to a perforating artery, 10 (35.7%) were due to a large vessel, 4 (14.3%) were venous, and 3 (10.7%) were related to hypoperfusion or embolic causes. Intraoperative MRI (iMRI) was used in 11 of the 28 cases, and 6 (55%) infarcts were not detected, all of which were deep.

CONCLUSIONS

The incidence of stroke following an elective craniotomy is low, with nearly all cases (86%) occurring after tumor resection. Perforator infarcts were most common but may be missed on iMRI.

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Matthew J. Zdilla

OBJECTIVE

The presence of a caroticoclinoid foramen may increase the likelihood of adverse neurosurgical events. Despite the clinical importance of the caroticoclinoid foramen, its study has been mostly limited to adult populations. Therefore, the object of this study was to describe the prevalence, morphology, and development of the caroticoclinoid foramen among varied sexes and races in early life.

METHODS

The study analyzed caroticoclinoid foramina in dry orbitosphenoid, presphenoid, and sphenoid bones from a population of 101 fetal and infantile crania of varied sex and race.

RESULTS

A caroticoclinoid foramen, whether complete, near complete, or partial, was found in 36 of 199 sides (18.1%). Of the 98 crania with bilaterally intact sides, 21 (21.4%) had the presence of at least one caroticoclinoid foramen. Caroticoclinoid foramina were found unilaterally and bilaterally, in both female and male crania (9/41, 22.0%; 12/57, 21.1%, respectively) and, likewise, in crania of both black and white races (9/54, 16.7%; 12/44, 27.3%, respectively). Caroticoclinoid foramina were formed from cornuate bony projections from the anterior clinoid process, middle clinoid process, or both anterior and middle clinoid processes. Caroticoclinoid foramina were also found in isolated orbitosphenoid bones from individuals as young as 4 months’ fetal age.

CONCLUSIONS

The caroticoclinoid foramen occurs in approximately one of every 5 sides and in one in every 5 individuals of perinatal age and should, therefore, be considered a common finding in both fetuses and infants. It is common in both females and males as well as in both black and white races, alike. Furthermore, the caroticoclinoid foramen can be found in individuals as young as 4 months of fetal age. Failure to anticipate the presence of a caroticoclinoid foramen will place important neurovascular structures, including the internal carotid artery, at risk of injury. Neurosurgeons should, therefore, anticipate the caroticoclinoid foramen even in their youngest patients.

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Jeffrey L. Nadel, D. Andrew Wilkinson, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

OBJECTIVE

The goal of this study was to determine the rates of screening and surgery for foramen magnum stenosis in children with achondroplasia in a large, privately insured healthcare network.

METHODS

Rates of screening and surgery for foramen magnum stenosis in children with achondroplasia were determined using de-identified insurance claims data from a large, privately insured healthcare network of over 58 million beneficiaries across the United States between 2001 and 2014. Cases of achondroplasia and screening and surgery claims were identified using a combination of International Classification of Diseases diagnosis codes and Current Procedural Terminology codes. American Academy of Pediatrics (AAP) practice guidelines were used to determine screening trends.

RESULTS

The search yielded 3577 children age 19 years or younger with achondroplasia. Of them, 236 met criteria for inclusion in the screening analysis. Among the screening cohort, 41.9% received some form of screening for foramen magnum stenosis, whereas 13.9% of patients were fully and appropriately screened according to the 2005 guidelines from the AAP. The screening rate significantly increased after the issuance of the AAP guidelines. Among all children in the cohort, 25 underwent cervicomedullary decompression for foramen magnum stenosis. The incidence rate of undergoing cervicomedullary decompression was highest in infancy (28 per 1000 patient-years) and decreased with age (5 per 1000 patient-years for all other ages combined).

CONCLUSIONS

Children with achondroplasia continue to be underscreened for foramen magnum stenosis, although screening rates have improved since the release of the 2005 AAP surveillance guidelines. The incidence of surgery was highest in infants and decreased with age.