Journal of Neurosurgery: Pediatrics
Volume 28: Issue 6 (Dec 2021)

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Benjamin M. Krainin, Corrine N. Seehusen, Katherine L. Smulligan, Mathew J. Wingerson, Julie C. Wilson, and David R. Howell

In Brief

Researchers examined the association between physical activity after a sport-related concussion and a set of multimodal, clinically oriented outcomes. Patients who reported engaging in early physical activity reported less symptom frequency and had a lower incidence of persistent postconcussion symptoms compared with patients who reported no early physical activity. These findings were in agreement with prior observations related to the potential benefit of activity after concussion, but further investigation is required to identify casual effects.

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Julian Zipfel, Juliane Engel, Konstantin Hockel, Ellen Heimberg, Martin U. Schuhmann, and Felix Neunhoeffer
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Pious D. Patel, Katherine A. Kelly, Heidi Chen, Amber Greeno, Chevis N. Shannon, and Robert P. Naftel
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Franz E. Babl, Vanessa C. Rausa, Meredith L. Borland, Amit Kochar, Mark D. Lyttle, Natalie Phillips, Yuri Gilhotra, Sarah Dalton, John A. Cheek, Jeremy Furyk, Jocelyn Neutze, Silvia Bressan, Gavin A. Davis, Vicki Anderson, Amanda Williams, Ed Oakley, Stuart R. Dalziel, Louise M. Crowe, and Stephen J. C. Hearps

In Brief

The aim of this study was to describe the age-related signs, symptoms, and epidemiology of concussion presentations to the emergency department in children by using a large multicenter prospective observational data set. Signs and symptoms of concussion varied by age, with higher levels of vomiting in younger children and higher levels of loss of consciousness and amnesia in older children. Concussions were increasingly sports related as age increased. The study illuminates age-related signs and symptoms of concussion and the epidemiology of concussion based on patient age.

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Vijay M. Ravindra, Sungho Lee, David Gonda, Ilana Patino, Lucia Ruggieri, Daniel S. Ikeda, and Daniel J. Curry

In Brief

This single-center case series has illustrated the use of MR-guided laser interstitial thermal therapy for the treatment of periventricular nodular heterotopia (PVNH)–related epilepsy in a pediatric population. The minimally invasive technique has met the need for deep treatment of the occasionally deep-seated seizure onset zones found in PVNH and avoids transventricular resection. Palliative outcomes were achieved in 4 of the 5 patients studied, without unexpected complications and with short hospital stays.

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Joseph H. Garcia, Ethan A. Winkler, Ramin A. Morshed, Alex Lu, Simon G. Ammanuel, Satvir Saggi, Elaina J. Wang, Steve Braunstein, Christine K. Fox, Heather J. Fullerton, Helen Kim, Daniel L. Cooke, Steven W. Hetts, Michael T. Lawton, Adib A. Abla, and Nalin Gupta

In Brief

Children with arteriovenous malformations (AVMs) usually present with either seizures or hemorrhage. The factors that lead to seizures are not well understood. Based on univariate analysis, frontal lobe location, larger AVM size, higher Supplemental Spetzler-Martin grade, and older age correlated with seizure presentation. Children with AVMs with these features should be considered at higher risk for seizures.

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Jacob R. Lepard, Irene Kim, Anastasia Arynchyna, Sean M. Lew, Robert J. Bollo, Brent R. O’Neill, M. Scott Perry, David Donahue, Matthew D. Smyth, and Jeffrey Blount

In Brief

The authors sought to evaluate the combined early experience of multiple pediatric stereoelectroencephalography (SEEG) centers. On average, patients received 11 electrodes and 7 days of monitoring. Subsequent therapeutic intervention occurred in 82% of procedures with approximately half of patients achieving seizure freedom. The majority of literature surrounding pediatric SEEG has been based on single-center, large-volume case series. This study has provided a more generalizable perspective in the learning curve and early implementation of pediatric SEEG.

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David C. Lauzier, Ananth K. Vellimana, Arindam R. Chatterjee, Joshua W. Osbun, Christopher J. Moran, Gregory J. Zipfel, and Akash P. Kansagra

In Brief

Investigators performed a retrospective cohort study and meta-analysis of angiographically cured pediatric arteriovenous malformations (AVMs) to quantify the risk of recurrence and identify risk factors influencing the rate of recurrence. Recurrence rates of 10.4% and 4.8% were identified in an institutional cohort and meta-analysis, respectively. Younger patient age was associated with higher rate of recurrence, while radiosurgical treatment was associated with lower rate of recurrence. These findings may inform angiographic follow-up of pediatric AVMs.

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Risheng Xu, Michael E. Xie, Wuyang Yang, Philippe Gailloud, Justin M. Caplan, Christopher M. Jackson, Eric M. Jackson, Mari L. Groves, Shenandoah Robinson, Alan R. Cohen, Judy Huang, and Rafael J. Tamargo

In Brief

Researchers summarized their experience over 30 years treating pediatric cerebral aneurysms. Of 4500 total patients with 5150 intracranial aneurysms, 47 children presented with 53 aneurysms. Pediatric aneurysms were more likely giant, in males, and within the MCA. The mortality rate was 10.6%. Of treated aneurysms, 6.7% recurred in the endovascular group, while none recurred in the microsurgical group. Treatment of pediatric aneurysms should be definitive, as the risk for recurrence will be managed over a lifetime.

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Mehmet N. Cizmeci, Linda S. de Vries, Maria Luisa Tataranno, Alexandra Zecic, Laura A. van de Pol, Ana Alarcon, Floris Groenendaal, and Peter A. Woerdeman
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Rebecca A. Reynolds, Ranbir Ahluwalia, Vishal Krishnan, Katherine A. Kelly, Jaclyn Lee, Raymond P. Waldrop, Bradley Guidry, Astrid C. Hengartner, Justin McCroskey, Anastasia Arynchyna, Susan Staulcup, Heidi Chen, Todd C. Hankinson, Brandon G. Rocque, Chevis N. Shannon, and Robert Naftel

In Brief

An estimated 10%–20% of all pediatric shunt malfunctions are associated with no radiographic findings and are often a diagnostic dilemma. In this large multicenter case-control study, the authors sought to identify risk factors for patients with shunted hydrocephalus who present without evidence of radiographic failure. Those children with a frontal shunt, programmable valve, nonsiphoning shunt, baseline large ventricles, and no prior shunt infection were more likely than other children with hydrocephalus to have unchanged ventricles at shunt failure.

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James Ulrich, John Caird, and Darach Crimmins

In Brief

In this study the authors sought to discover variables that could aid in predicting outcomes in patients with Dandy-Walker malformation (DWM). The data show that the presence of other anomalies of the CNS and larger cyst size predicted worse outcomes. These results are simultaneously in contrast to some previous research and further develop ideas revealed in other studies. This research was conducted in the hopes of better advising parents of children with DWM.

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Christopher L. Kalmar, Zachary D. Zapatero, Mychajlo S. Kosyk, Anna R. Carlson, Scott P. Bartlett, Gregory G. Heuer, Alexander M. Tucker, Jesse A. Taylor, Shih-Shan Lang, and Jordan W. Swanson

In Brief

Researchers measured intracranial pressure in subjects undergoing treatment of craniosynostosis, and created a multivariate model to assess factors that are associated with elevated intracranial pressure. Increased age, increased number of involved sutures, and an associated craniofacial syndrome were each independently linked to risk of elevated intracranial pressure. These findings may have implications for risk factors and optimal treatment strategies for craniosynostosis.

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Fritz Teping, Stefan Linsler, Michael Zemlin, and Joachim Oertel
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Andy S. Ding, Sakibul Huq, Joshua Casaos, Divyaansh Raj, Manuel Morales, Tianna Zhao, Timothy Kim, Siddhartha Srivastava, Ayush Pant, Riccardo Serra, Noah L. Gorelick, Henry Brem, and Betty Tyler

In Brief

The authors evaluated the efficacy of TG02, a novel multi–CDK inhibitor, in atypical teratoid rhabdoid tumors (ATRTs), a group of aggressive pediatric CNS malignancies. TG02 was found to inhibit proliferation and induce apoptosis in multiple ATRT cell lines, while also potentiating the antitumor effects of radiation and cisplatin. These findings have positioned TG02 as a promising adjuvant therapeutic that can decrease the necessary dose of chemoradiation in patients with ATRTs, thereby mitigating systemic side effects associated with treatment.

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Nishanth Sadashiva, Andiperumal Raj Prabhuraj, and Bhagavatula Indira Devi
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Daniel H. Fulkerson and Jodi L. Smith
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In Brief

The authors investigated the clinical efficacy of endoscopic transcortical intraventricular biopsy for isolated pituitary stalk lesions in children. In this series, the diagnostic yield was 93.8%. Of the 16 patients, 2 patients developed new-onset diabetes insipidus after the procedure, and 3 had new-onset postoperative hypothyroidism. There have been no postoperative neurological deficits. Neuroendoscopic biopsy via the transcortical intraventricular corridor is safe and can aid in a reliable histopathological diagnosis for isolated pituitary stalk lesions.

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In Brief

Single-dose and multiple-dose prophylactic antibiotic usage in cranial and spinal neurosurgical interventions of pediatric patients were compared through a propensity-matched study. There was no difference between the two in clean spinal surgery and instrumented and noninstrumented cranial surgery with regard to postoperative infection. Use of single-dose cephalosporin was effective in such circumstances and, considering the benefits of lower administration and potential reduction of dose-dependent adverse effects, is a matter that needs further investigation.

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In Brief

The purpose of the study was to determine the social determinants that influence access and outcomes for pediatric neurosurgical care for pediatric patients with Chiari malformation type I and syringomyelia. Race was associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for all patients with Chiari malformation type I and syringomyelia.

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In Brief

In this study, pediatric patients with Chiari malformation type I were assessed for the effect of socioeconomic factors and race on surgical treatment. Non-White patients were more likely to be socioeconomically disadvantaged, although race and disadvantage were not associated with undergoing surgical treatment. Patients with benign Chiari malformations undergoing decompression for delayed symptom presentation resided in more affluent and urban areas. This study points to socioeconomic disparities in the care of pediatric Chiari malformations.

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Routine imaging after ventriculoperitoneal shunt revision is relatively common, but it is unclear whether it leads to clinical interventions. Researchers examined a large cohort of patients who underwent shunt revision to determine the utility of postoperative imaging. In only 0.8% of revisions, postoperative imaging led to a change in management that was not otherwise heralded by clinical concerns, indicating a low utility for routine imaging.

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The authors report the neurodevelopmental outcomes of 41 patients from a single institution diagnosed with AVID (asymmetric ventriculomegaly, interhemispheric cyst, and dysgenesis of the corpus callosum) before birth. From our data, it appears that the neurologic and developmental outcomes of these children, while still guarded, are better than previously reported. These additional data may assist us in future prenatal counseling of families with a fetal diagnosis of AVID.

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The authors investigated predictors of posthemorrhagic ventricular dilatation in a porcine model using direct injection of untreated autologous blood. Clot volume and degree of ventriculomegaly on immediately postoperative MRI, rather than amount of blood injected, predicted sustained ventriculomegaly. These findings suggest that clot formation and early disruption of spinal fluid flow may have a more significant contribution to subsequent development of hydrocephalus than the mere presence of ventricular blood.

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Pediatric patients with a lipoma of the conus medullaris (LCM) who underwent untethering surgery using the partial removal technique were retrospectively reviewed to evaluate long-term surgical outcomes. A lipoma type of lipomyelomeningocele and a high patient growth rate postoperatively were shown to be significant factors associated with postoperative ReTCS in pediatric LCMs. To perform prophylactic surgery in infancy for LCMs with a high risk of postoperative adhesion may not be a good option.

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In Brief

The Hydrocephalus Clinical Research Network conducted a prospective cohort study of school-aged children with new hydrocephalus to determine if the treated ventricle size 6 months after surgery was associated with functional outcomes. Quality of life, school performance, and 23 of 25 neuropsychological tests, including full-scale IQ, were not associated with ventricle size. These findings have suggested that the resultant ventricle size after successful CSF diversion plays little role in predicting short-term functional outcomes in children with hydrocephalus.

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The objective of this study was to determine the various complications of neuroendoscopic septostomy and their incidence rates. Compared with endoscopic third ventriculostomy/choroid plexus cauterization, septostomy for other indications did not appear to harbor additional risk. In this large series, ventricular size, morphology, and other intraoperative factors did not affect the rate of complications.

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In Brief

Investigators reviewed a large cohort of children who underwent cerebral angiography to rigorously evaluate complication rates and risk factors. Complications occurred in 6.5% of procedures, including neurological complications in 1.9% and nonneurological complications in 4.8%. Most complications were transient. Female sex and a history of hypertension or ischemic stroke were associated with increased risk of complications. These findings may facilitate clinical discussions of risks and benefits of cerebral angiography in children.

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An international working group of female pediatric neurosurgeons summarized the status and challenges of women in pediatric neurosurgery worldwide. Unmet needs were found to include board certification, the recognition of pediatric neurosurgery as a subspecialty, a lack of female pioneers, and academic advancement. The present study has provided significant data and an international perspective to address advancements for women in this field.

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In Brief

The authors sought to study predictors for early posttraumatic seizures (EPTSs) in children with moderate to severe traumatic brain injury (TBI), the association between EPTSs and death, and the association between EPTSs and poor functional outcomes. EPTSs occurred more commonly in younger children with TBI and were associated with poor functional outcomes among children who survived TBI. The authors’ findings have validated those of other single-center studies on the association between EPTSs and poor outcomes in children with TBI.

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The authors have enumerated all previously reported high-confidence congenital hydrocephalus risk genes, as well as reviewed the history of gene discovery and particular phenotypic findings for each gene. Additionally, they have provided evidence of an increasing gene discovery rate as sequencing technologies become increasingly more accessible, the multifold benefits of genetic diagnosis, and the necessity of further gene discovery and functional investigation to improve clinical understanding. Incorporating genomic investigation into the workup of all patients with complex congenital hydrocephalus is recommended.

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Predicting the outcomes of posterior fossa decompression for pediatric patients with Chiari I malformation type (CM-I) hinges on the availability of reliable and holistic outcome assessment tools. The objective of this study was to incorporate patient-centered quality-of-life instruments in an outcome assessment. As measured with the Patient-Reported Outcomes Measurement Information System (PROMIS) tool, a pilot cohort showed moderate improvement of health-related quality of life after surgery. This report exemplifies the utility of patient-centered metrics in the evaluation of surgical treatment of pediatric patients with CM-I.

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The authors sought to evaluate the natural history and pathological spectrum of incidental thalamic tumors in children from eight pediatric centers. The key finding was that thalamic incidentalomas in children included mostly low-grade lesions; however, high-grade lesions may also present as incidentalomas. Thus, radiological follow-up of lesions is mandatory, as even lesions with a typical low-grade appearance may evolve over time.

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Researchers investigated variables associated with postoperative seizures following endoscopic third ventriculostomy and choroid plexus cauterization (ETV/CPC) for treatment of pediatric hydrocephalus. The most significant finding was that 0% (0 of 18) of patients who received prophylactic perioperative levetiracetam experienced postoperative seizures compared with 47% (9 of 19) of those who did not, indicating perioperative levetiracetam may be efficacious in preventing postoperative seizures. This study is the first to include EEG data showing that seizures may be associated with endoscope placement.

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In this study, the authors sought to quantify the burden of pediatric neurosurgical disease in a rural developing country. Pediatric neurosurgical disease, predominantly trauma and congenital anomalies, accounted for 20.4% of the total neurosurgical needs at a single center in Nigeria. The study results provides insight into the burden of pediatric neurosurgical disease and the barriers to neurosurgical care in a low-middle-income country.

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Researchers investigated the dosimetric parameter associated with long-term control of sellar and parasellar tumors after Gamma Knife surgery. D98% was significantly associated with long-term tumor control, with D98% ≥ 10 Gy being necessary for craniopharyngiomas. The study results have shown that D98% would be a reliable index of the minimum required dose for long-term control of sellar and parasellar tumors in proximity to the optic apparatus, helping clinicians prescribe the proper effective dose for tumor control while maintaining safety, especially in regard to radiation-induced optic neuropathy.

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The underlying biomechanical differences between the pediatric and adult cervical spine are incompletely understood. Computational spine modeling, like finite element modeling (FEM), can address that knowledge gap. A pediatric cervical spine FEM was created that accurately predicts axial tension and flexion-extension range of motion when ligamentous and annulus material properties are reduced to 50% of published adult properties. This model shows promise for use in surgical simulation procedures and as a normal comparison for disease-specific FEMs.

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In this systematic review, the authors sought to review the published evidence on the use of cranial neuromodulation—deep brain stimulation (DBS) and responsive neurostimulation (RNS)—for pediatric drug-resistant epilepsy (DRE). From 35 studies, they found 72 children who had undergone DBS and 42 who had undergone RNS. Overall, 75% and 73.2% of DBS and RNS patients, respectively, experienced > 50% reduction in seizures. This study shows the paucity of evidence for promising treatment options for pediatric DRE.

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Researchers applied MRI to explore conus medullaris (CM) migration in early infancy. The CM of term infants reached the normal adult level at birth–i.e., on average between the L1–2 intervertebral disc and the inferior border of the L2 vertebra–and did not ascend during childhood. The postnatal migration process of CM remains unclear, and MRI has yet to be used to investigate the normal CM level in early infancy.

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In the population with nonsyndromic single-suture craniosynostosis, the authors compared the quality of life for those treated with surgery versus those treated conservatively. A high number of children received conservative management. No independent association was found between management type and quality of life when adjusted for confounding patient factors. Surgeons may be having less effect on the outcomes important to patients than previously realized, highlighting the urgency for further research to build off of these findings.

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In Brief

This study aimed to characterize pediatric cranial neurotrauma resulting from motorized recreational vehicle crashes and the role of helmets in injury prevention. Helmeted pediatric riders had significantly reduced odds of intracranial injury and moderate or severe traumatic brain injury and much less frequently required a neurosurgical consultation or procedure than unhelmeted patients. This study makes clear the role of helmets in neurologic injury prevention and suggests an enormous opportunity for collaboration between neurosurgeons and injury prevention professionals.

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Researchers evaluated the RNF213 p.R4810K variant and clinical information of 129 Japanese patients with pediatric-onset moyamoya disease. Compared with the patients with the heterozygous variant, patients without the p.R4810K variant were younger at disease onset and greater proportions had infarction at onset and on initial imaging. All patients who presented at less than 1 year of age lacked the p.R4810K variant. The absence of the RNF213 p.R4810K variant may be a novel biomarker for identification of a severe form of pediatric moyamoya disease.

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Malignant peripheral nerve sheath tumors are very rare in pediatric patients. The objective of this study was to describe how these tumors behave clinically in this population. The authors showed that there was a clear survival benefit in pursuing maximal safe resection in pediatric patients, with the roles of chemotherapy and radiation therapy not well established. Given the large cohort derived from a national database, these findings are the most statistically robust to date.

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The authors studied a nationally derived cohort of pediatric patients who underwent shunt insertion for hydrocephalus from 2016 to 2019 to determine risk factors for early failure and infection. Overall, 7.3% of shunt insertions resulted in unplanned revision within 30 days. Hydrocephalus etiology, age, and presence of ostomy were important predictors of the need for early shunt revision. These findings could be used for benchmarking in hospital efforts to improve quality of care for pediatric patients with hydrocephalus.

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The objective of this study was to test whether a new interinstitutional, multimodal presurgical evaluation protocol was associated with improved seizure freedom for poorly defined cases of focal epilepsy in children. The new systematic protocol, using advanced neuroimaging and electrophysiology tests, was associated with a 1-year postsurgical seizure freedom rate of 91% versus 47% in the preprotocol era. This is a major step forward in improving the care of children with poorly defined focal epilepsy.

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Researchers reviewed the survival of ventricular access devices (VADs) used for drug delivery (cerliponase alfa) in patients with late infantile neuronal ceroid lipofuscinosis type 2. The median survival of newly inserted VADs was 2317 days. The main reason for revision was suspected infection. Such information is valuable when counseling patients and their families. Given the current interest in intrathecal drug delivery for rare metabolic disorders, the need for VADs is likely to increase.

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The authors aimed to identify whether noninvasive arterial spin labeling (ASL) can predict postoperative angiogram results following pial synangiosis surgery in children. Patients with ASL imaging without digital subtraction angiography (DSA) had similar postoperative outcomes when compared with those with ASL imaging and postoperative DSA. Noninvasive ASL perfusion imaging had an association with postoperative DSA neoangiogenesis. This study supports movement in the direction of validating noninvasive imaging modalities in lieu of invasive catheter angiography.

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This study characterized subdural collections in African infants with postinfectious hydrocephalus following surgical treatment. Using normalized volumetrics for brain and CSF, higher preoperative CSF volume was associated with both higher rates and larger volumes of subdural accumulation. The presence and size of the subdural collection were associated with lower cognitive scores. These results suggest that preoperative CSF volumes could be used for risk stratification for treatment decision-making and for future clinical trials of alternative shunt technologies.

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Researchers utilized a novel photograph image-processing technique to evaluate changes in key craniometrics over time among a set of patients with metopic and sagittal craniosynostoses following endoscopic surgical treatment. Metopic patients demonstrated a longer interval of measurable deformity improvement during postoperative molding helmet therapy than sagittal craniosynostosis patients. This information suggests that metopic patients may benefit from longer periods of helmet therapy than sagittal craniosynostosis patients.