Heather J. McCrea, Jana Ivanidze, Ashley O’Connor, Eliza H. Hersh, John A. Boockvar, Y. Pierre Gobin, Jared Knopman, and Jeffrey P. Greenfield
The authors assessed the safety of intraarterial delivery of bevacizumab and cetuximab in pediatric patients with high-grade glioma or diffuse intrinsic pontine glioma (DIPG) to increase drug delivery across the blood-brain barrier. There were no dose-limiting toxicities. Six of 10 patients with symptoms had improvement. The mean overall survival for 10 DIPG patients was 17.3 months. This study demonstrates the safety of this delivery method, now allowing for testing repeat dosing and additional agents to hopefully increase drug delivery and efficacy.
Alexandre Roux, Kévin Beccaria, Thomas Blauwblomme, Nizar Mahlaoui, Fabrice Chretien, Pascale Varlet, Stéphanie Puget, and Johan Pallud
The authors describe the joint collaboration of the Necker-Enfants Malades and the Sainte-Anne Hospital for transitional care in surgical neurooncology. They suggest that neurosurgeons involved in transitional care should combine both pediatric and adult surgical neurooncology skills to optimize care management of these patients within a dedicated multidisciplinary organization framework. This study highlights the importance of organizing a transitional care in surgical neurooncology to improve patient care.
Robert C. Rennert, Michael G. Brandel, Shanmukha Srinivas, Divya Prajapati, Omar M. Al Jammal, Nolan J. Brown, Luis D Diaz-Aguilar, Jennifer Elster, David D. Gonda, John R. Crawford, and Michael L. Levy
The authors aimed to report their experience performing palliative endoscopic third ventriculostomy (ETV) in pediatric patients for hydrocephalus secondary to unresectable primary brain tumors. The performance of a palliative ETV in this population is safe (only one minor perioperative complication) and successful (as determined by avoidance of a secondary shunt) in nearly 50% of patients (7 of 15). This work provides original data on the utility of palliative ETV in pediatric patients with hydrocephalus secondary to unresectable primary brain tumors.
The authors assessed the benefit of early pediatric epilepsy surgery on developmental outcomes. Among 75 patients who had undergone a first curative epilepsy surgery under the age of 3 years, seizure freedom was achieved in 82.7% at 1 year after surgery and postoperative complications requiring shunting surgery were observed in 17.3%. The 1-year postoperative developmental quotient (DQ) was influenced by preoperative DQ and postoperative seizure freedom but not by the occurrence of surgical complications. This study revealed that seizure control after surgery was important for postoperative development.
Ioannis N. Mavridis, William B. Lo, Welege Samantha Buddhika Wimalachandra, Sunny Philip, Shakti Agrawal, Caroline Scott, Darren Martin-Lamb, Bryony Carr, Peter Bill, Andrew Lawley, Stefano Seri, and A. Richard Walsh
Authors of this study assessed stereo-electroencephalography (SEEG) safety in the pediatric setting, focusing on surgical complications, and explored whether robot assistance was associated with a change in practice, surgical parameters, and clinical outcomes. SEEG complications are uncommon and usually transient or treatable. Robot assistance enabled implantation of more electrodes and improved epilepsy surgery outcome compared to those in non—robot-assisted cases. This is the largest single-center, exclusively pediatric SEEG series that includes robot assistance so far.
Shih-Shan Lang, Petra M. Meier, William Z. Paden, Phillip B. Storm, Gregory G. Heuer, Scott P. Bartlett, Jesse A. Taylor, Jordan W. Swanson, John G. Meara, Mark Proctor, Oluwatimilehin Okunowo, Paul A. Stricker, and on behalf of the Pediatric Craniofacial Collaborative Group
Both spring-mediated cranioplasty (SMC) and endoscopic strip craniectomy (ESC) have been associated with favorable perioperative outcomes as compared to those with open vault reconstruction. However, there are limited data comparing perioperative outcomes between SMC and ESC. Using a large prospective multicenter registry, authors of this study compared perioperative outcomes between these two minimally invasive approaches. The study findings revealed similar perioperative transfusion outcomes, with SMC insertion procedures having higher ICU admission rates and longer hospital stays.
Catherine A. de Planque, Steven A. Wall, Louise Dalton, Giovanna Paternoster, Éric Arnaud, Marie-Lise C. van Veelen, Sarah L. Versnel, David Johnson, Jayaratnam Jayamohan, and Irene M. J. Mathijssen
Clinical signs in relation to the required interventions and treatment course of patients with Crouzon syndrome with acanthosis nigricans have been assessed at three craniofacial centers. Patients with the c.1172C>A (p.Ala391Glu) mutation in the FGFR3 gene follow a severe disease trajectory, with numerous interventions. The timing and order of interventions differed among patients and centers. This study is a first effort to collect data from multiple centers on a rare form of complex craniosynostosis.
Jacob Lepard, S. Hassan A. Akbari, James Mooney, Anastasia Arynchyna, Samuel G. McClugage III, René P. Myers, John Grant, and James M. Johnston Jr.
In this study the authors compare subjective ratings of aesthetic outcomes between open and endoscopic management of isolated sagittal synostosis by using standardized photography. The mean rating for the endoscopic group was significantly better than that for the open repair group. This relationship remained true for raters with a variety of medical training backgrounds. This study provides pragmatic insight into variations in aesthetic outcomes based on the method of surgical correction.
Through a systematic review and meta-analysis, the authors compared the use of rigid and flexible endoscopes in endoscopic third ventriculostomy (ETV) with or without choroid plexus cauterization (±CPC). They found similar ETV success and complication rates for ETV±CPC with flexible versus rigid endoscopy, despite lower calculated ETV success scores for patients treated with flexible endoscopy. The choice of surgical instrument has not previously been directly compared and may warrant further study.
Eisha A. Christian, Jeffrey J. Quezada, Edward F. Melamed, Carolyn Lai, and J. Gordon McComb
The authors determined the outcome of using the pleural space as the terminus for ventricular CSF-diverting shunts in children. Ventriculopleural shunts in children younger than 10 years had significantly higher rates of symptomatic pleural effusion requiring revision to a different location. In patients older than 10 years, all else being equal, the authors recommend that the distal end of the shunt be placed into the pleural space rather than the right atrium if the peritoneal cavity is not suitable.
Mallory R. Peterson, Venkateswararao Cherukuri, Joseph N. Paulson, Paddy Ssentongo, Abhaya V. Kulkarni, Benjamin C. Warf, Vishal Monga, and Steven J. Schiff
The authors created normative childhood brain growth curves with percentiles for clinical use. Brain volume peaked at 10–12 years, and males exhibited larger age-adjusted total brain volumes than females despite body size normalization. However, the authors unexpectedly discovered that the brain has a universal age-dependent relationship to cerebrospinal fluid independent of sex or body size. Normative brain growth curves will be of value in managing childhood disease in which cognitive development, brain growth, and fluid accumulation are interrelated.
Zachary Spiera, Theodore Hannah, Adam Li, Nickolas Dreher, Naoum Fares Marayati, Muhammad Ali, Dhruv S. Shankar, John Durbin, Alexander J. Schupper, Alex Gometz, Mark Lovell, and Tanvir Choudhri
Concussion prevention has become a major focus for amateur sports leagues. The authors aimed to assess the effects of baseline preinjury nonsteroidal anti-inflammatory drug (NSAID) use on susceptibility to concussion in adolescent athletes. The study data provide evidence that the preinjury use of NSAIDs does not affect concussion incidence, severity, or recovery in this population. To the authors' knowledge, this study is the first to directly assess the effects of preinjury anti-inflammatory drug use on concussion risk in adolescents.
William B. Lo, Katie Herbert, and Desiderio Rodrigues
The effectiveness of and families' experiences with telephone consultation (TC) were evaluated. Of 114 children, none required a face-to-face (FTF) appointment within 3 months after TC. For 89% of families, a TC was the same/better than an FTF appointment. Families attending TCs for their initial appointments preferred the next appointment to be FTF. A preference for the next appointment to be a TC was associated with a better current TC experience and longer home-to-hospital distance. New patients should be offered FTF appointments and follow-up TC when possible.
Gad Dotan, Natalie Hadar Cohen, Hanya M. Qureshi, Mika Shapira Rootman, Yoram Nevo, and Amir Kershenovich
This study assessed the effectiveness of external lumbar drainage in children with idiopathic intracranial hypertension and worsening papilledema despite medications for lowering intracranial pressure. Performed early when vision is preserved, this intervention typically results in rapid and sustainable resolution of patients’ symptoms and papilledema with a good visual outcome, suggesting that it may be considered as second-stage therapy in this setting, after failed medical therapy, before proceeding to definitive surgeries that carry inherent risks and failures.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Researchers investigated possible factors predicting permanent shunt conversion in a cohort of preterm infants treated for posthemorrhagic hydrocephalus with temporary external ventricular drainage. The shunt-dependent infants had a significantly lower gestational age, but no other clinical or MRI neuroradiological features significantly differed between the shunt-free and shunt-dependent infants. This study details the external ventricular drainage technique and its role as initial treatment for hydrocephalus in preterm infants.
This study investigated the practice patterns of pediatric neurosurgeons around the world who care for occult spinal dysraphism, also known as spinal cord tethering. The survey data have shown consensus on the need to image children with dramatic lumbar cutaneous findings such as a fatty mass and vestigial tail. Management practices varied considerably on more common cutaneous stigmata including a sacral dimple. Management varied substantially on when to offer prophylactic surgery for a tethered cord.
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.
Investigators applied lesion network mapping to children with focal cortical dysplasia who underwent surgery for drug-resistant epilepsy. Lesion volumes with persistent seizures after surgery tended to have stronger connectivity to attention and motor networks and weaker connectivity to the default mode network, compared with lesion volumes with seizure-free surgical outcomes. Network connectivity–based lesion-outcomes mapping may offer new insight for determining the impact of lesion volumes discerned according to both size and specific location.
The goal of this paper was to describe a neurosurgical educational partnership and the early results in developing pediatric craniofacial expertise in Southeast Asia. After brief, focused training through partnership with an institution in the United States followed by consistent telecollaboration, Vietnamese neurosurgical colleagues were able to rapidly scale up treatment of craniosynostosis with good outcomes. Global neurosurgical partnerships are feasible and effective methods of education and development of local neurosurgical expertise in low- and middle-income countries.
Social determinants of health, such as race, are infrequently reported and/or considered in the sport-related concussion (SRC) literature. Using a retrospective registry of SRC patients, investigators compared the recovery experiences of White and Black athletes. Black athletes reported earlier symptom resolution and return to school and were less likely to report a change to their daily activities than White athletes. These findings suggest that clinicians and researchers need to consider social determinants of health more closely in SRC.
Researchers report three cases of primary malignant spindle cell tumors in the posterior fossa of children and performed a literature review pertaining to management strategies for these entities. These lesions are extremely rare neoplasms that appear to respond to multimodal therapy using resection, radiotherapy, and chemotherapy. The full clinical significance of using multiple treatment modalities for these rare extraaxial mesenchymal tumors is yet to be determined, but multimodal therapy may effectively reduce the mortality rate in the pediatric population.
Researchers administered the Behavior Assessment System for Children, Third Edition (BASC-3) and the Hydrocephalus Outcome Questionnaire (HOQ) to caregivers of children with hydrocephalus. Children with hydrocephalus of all etiologies had more difficulties with social-emotional functioning compared with normative populations. The social-emotional domain of the HOQ correlated more strongly with the BASC-3 than did the physical and cognitive domains, demonstrating its utility as a brief clinic screener to identify patients potentially in need of behavioral healthcare.
Amid budget crises, cutting costs while maintaining quality care is a top priority. The authors hypothesized that by instituting a standardized postoperative care protocol for Chiari malformations, hospital length of stay and cost to families and hospital systems would decrease. A unified protocol was found to decrease the length of stay, healthcare costs, and readmissions while maintaining high quality and patient safety. Similar unified postoperative care plans could target other common neurosurgical interventions to improve care and decrease healthcare costs.
The objective of this cohort study was to describe and analyze the surgical treatment and outcome of posterior fossa arachnoid cysts (PFACs) in infants. Clinical and radiological outcomes were good in all patients, although in about 30%, revision surgery due to recurrent or persisting symptoms caused by the PFAC or hydrocephalus, was needed. Around 50% of the treated retrocerebellar cysts were surgically revised. Revision surgery was not needed in any of the treated supracerebellar cysts. Patients who were treated before the age of 6 months and who presented before surgery with a stable cyst size showed a trend towards higher revision rates after surgical treatment. This is the first study focusing specifically on PFACs in infants, their surgical treatment and outcome, as well as factors influencing outcome.
The authors analyzed the time course of coagulation and fibrinolytic parameters in the acute phase of traumatic brain injury in pediatric and adult patients to identify the relationship between coagulation and fibrinolytic parameters and age. More prolonged prothrombin time– international normalized ratio and activated partial thromboplastin time within 12 hours and lower fibrinogen concentrations within 6 hours after injury were observed in pediatric patients compared with adult patients, while there was no difference in D-dimer levels. However, a high D-dimer level was a negative prognostic factor.