Browse

You are looking at 21 - 30 of 2,373 items for :

  • Journal of Neurosurgery: Pediatrics x
  • Refine by Access: all x
Clear All
Full access

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.

Full access

Manish Kuchakulla, Ashish H. Shah, Valerie Armstrong, Sarah Jernigan, Sanjiv Bhatia, and Toba N. Niazi

OBJECTIVE

Carotid body tumors (CBTs), extraadrenal paragangliomas, are extremely rare neoplasms in children that often require multimodal surgical treatment, including preoperative anesthesia workup, embolization, and resection. With only a few cases reported in the pediatric literature, treatment paradigms and surgical morbidity are loosely defined, especially when carotid artery infiltration is noted. Here, the authors report two cases of pediatric CBT and provide the results of a systematic review of the literature.

METHODS

The study was divided into two sections. First, the authors conducted a retrospective review of our series of pediatric CBT patients and screened for patients with evidence of a CBT over the last 10 years (2007–2017) at a single tertiary referral pediatric hospital. Second, they conducted a systematic review, according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, of all reported cases of pediatric CBTs to determine the characteristics (tumor size, vascularity, symptomatology), treatment paradigms, and complications.

RESULTS

In the systematic review (n = 21 patients [includes 19 cases found in the literature and 2 from the authors’ series]), the mean age at diagnosis was 11.8 years. The most common presenting symptoms were palpable neck mass (62%), cranial nerve palsies (33%), cough or dysphagia (14%), and neck pain (19%). Metastasis occurred only in 5% of patients, and 19% of cases were recurrent lesions. Only 10% of patients presented with elevated catecholamines and associated sympathetic involvement. Preoperative embolization was utilized in 24% of patients (external carotid artery in 4 and external carotid artery and vertebral artery in 1). Cranial nerve palsies (cranial nerve VII [n = 1], IX [n = 1], X [n = 4], XI [n = 1], and XII [n = 3]) were the most common cause of surgical morbidity (33% of cases). The patients in the authors’ illustrative cases underwent preoperative embolization and balloon test occlusion followed by resection, and both patients suffered from transient Horner’s syndrome after embolization.

CONCLUSIONS

Surgical management of CBTs requires an extensive preoperative workup, anesthesia, and multimodal surgical management. Due to a potentially high rate of surgical morbidity and vascularity, balloon test occlusion with embolization may be necessary in select patients prior to resection. Careful thorough preoperative counseling is vital to preparing families for the intensive management of these children.

Full access

John D. Heiss, Aria Jamshidi, Smit Shah, Staci Martin, Pamela L. Wolters, Davis P. Argersinger, Katherine E. Warren, and Russell R. Lonser

OBJECTIVE

In this clinical trial report, the authors analyze safety and infusion distribution of IL13-Pseudomonas exotoxin, an antitumor chimeric molecule, administered via intratumoral convection enhanced delivery (CED) in pediatric patients with diffuse intrinsic pontine glioma (DIPG).

METHODS

This was a Phase I single-institution, open-label, dose-escalation, safety and tolerability study of IL13-PE38QQR infused via single-catheter CED into 5 pediatric DIPG patients. IL13-PE38QQR was administered to regions of tumor selected by radiographic findings. Two escalating dose levels were evaluated: 0.125 µg/mL in cohort 1 and 0.25 µg/mL in cohort 2. Real-time MRI was performed during intratumoral infusions, and MRI and MR spectroscopy were performed before and after the infusions. Clinical evaluations, including parent-reported quality of life (QOL), were assessed at baseline and 4 weeks post-infusion.

RESULTS

Direct infusion of brainstem tumor with IL13-PE using the CED technique in patients with DIPG produced temporary arrest of disease progression in 2 of 5 patients, both of whom subsequently received a second infusion. All 5 patients showed signs of disease progression by 12 weeks after initial infusion. Two patients experienced transient cranial nerve deficits and lethargy after infusion, and these deficits resolved with corticosteroid treatment in both cases. No patient had radiographic evidence of acute or long-term treatment toxicity. Parent-reported QOL was consistent with medical outcomes.

CONCLUSIONS

Even though IL13-PE delivered by CED did not reach the entire MRI-defined tumor volume in any patient, short-term radiographic antitumor effects were observed in 2 of the 5 patients treated. The patients’ performance status did not improve. Drug delivery using multiple catheters may produce improved outcomes.

Clinical trial registration no.: NCT00088061 (clinicaltrials.gov)

Full access

Robert A. McGovern, Elia Pestana Knight, Ajay Gupta, Ahsan N. V. Moosa, Elaine Wyllie, William E. Bingaman, and Jorge Gonzalez-Martinez

OBJECTIVE

The goal in the study was to describe the clinical outcomes associated with robot-assisted stereoelectroencephalography (SEEG) in children.

METHODS

The authors performed a retrospective, single-center study in consecutive children with medically refractory epilepsy who were undergoing robot-assisted SEEG. Kaplan-Meier survival analysis was used to calculate the probability of seizure freedom. Both univariate and multivariate methods were used to analyze the preoperative and operative factors associated with seizure freedom.

RESULTS

Fifty-seven children underwent a total of 64 robot-assisted procedures. The patients’ mean age was 12 years, an average of 6.4 antiepileptic drugs (AEDs) per patient had failed prior to implantation, and in 56% of the patients the disease was considered nonlesional. On average, children had 12.4 electrodes placed per implantation, with an implantation time of 9.6 minutes per electrode and a 10-day postoperative stay. SEEG analysis yielded a definable epileptogenic zone in 51 (89%) patients; 42 (74%) patients underwent surgery, half of whom were seizure free at last follow-up, 19.6 months from resection. In a multivariate generalized linear model, resective surgery, older age, and shorter SEEG-related hospital length of stay were associated with seizure freedom. In a Cox proportional hazards model including only the children who underwent resective surgery, older age was the only significant factor associated with seizure freedom. Complications related to bleeding were the major contributors to morbidity. One patient (1.5%) had a symptomatic hemorrhage resulting in a permanent neurological deficit.

CONCLUSIONS

The authors report one of the largest pediatric-specific SEEG series demonstrating that the modern surgical management of medically refractory epilepsy in children can lead to seizure freedom in many patients, while also highlighting the challenges posed by this difficult patient population.

Full access

Xiaolin Ai, Zengpanpan Ye, Jianguo Xu, Chao You, and Yan Jiang

OBJECTIVE

Rupture of arteriovenous malformations (AVMs) would result in high mortality and prevalence of disability in pediatric patients. Decisions regarding the treatment of AVMs need to weigh the risk of rupture over the course of their natural history against the possibility of creating a lesion during treatment. Multiple factors have been proposed to predict hemorrhagic presentation of pediatric patients with AVMs. The aim of this meta-analysis was to evaluate the predictors of hemorrhagic presentation in pediatric patients with AVMs.

METHODS

The authors searched the PubMed and EMBASE databases. Studies reporting the predictors of hemorrhagic presentation in children with untreated brain AVMs were included. The predictive ability of identified predictors was assessed by odds ratios (ORs) and 95% confidence intervals (CIs).

RESULTS

A higher risk of hemorrhagic presentation was found in AVMs with smaller size (< 3 cm, OR 2.97, 95% CI 1.94–4.54, p < 0.00001), deep venous drainage (OR 2.28, 95% CI 1.55–3.36, p < 0.0001), a single draining vein (OR 2.23, 95% CI 1.27–3.92, p = 0.005), a single feeder (OR 3.72, 95% CI 1.31–10.62, p = 0.01), a deep location (OR 1.82, 95% CI 1.22–2.72, p = 0.004), an infratentorial location (OR 2.25, 95% CI 1.19–4.26, p = 0.01), and diffuse morphology (OR 8.94, 95% CI 3.01–26.55, p < 0.0001). In addition, the AVMs with draining vein ectasia (OR 0.35, 95% CI 0.13–0.97, p = 0.04) and high Spetzler-Martin (SM) grade (OR 0.53, 95% CI 0.36–0.78, p = 0.001) had a lower risk of hemorrhagic presentation in pediatric patients.

CONCLUSIONS

Smaller AVMs, deep venous drainage, a single draining vein, a single feeder, deep/infratentorial location, diffuse morphology, and high SM grade were identified as positive predictors for hemorrhagic presentation. Particularly, patients with diffuse AVMs have a higher risk of hemorrhagic presentation than other factors and may need active treatments. However, factors such as age, sex, draining vein stenosis, and associated aneurysms were not associated with hemorrhagic presentation.

Free access

Victor M. Lu, Mohammed A. Alvi, Kerrie L. McDonald, and David J. Daniels

OBJECTIVE

Pediatric high-grade gliomas (pHGGs), including diffuse intrinsic pontine glioma, present a prognostic challenge given their lethality and rarity. A substitution mutation of lysine for methionine at position 27 in histone H3 (H3K27M) has been shown to be highly specific to these tumors. Data are accumulating regarding the poor outcomes of patients with these tumors; however, the quantification of pooled outcomes has yet to be done, which could assist in prioritizing management. The aim of this study was to quantitatively pool data in the current literature on the H3K27M mutation as an independent prognostic factor in pHGG.

METHODS

Searches of seven electronic databases from their inception to March 2018 were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Data were extracted and pooled using a meta-analysis of proportions. Meta-regression was used to identify potential sources of heterogeneity.

RESULTS

Six observational studies satisfied the selection criteria for inclusion. They reported the survival outcomes of a pooled cohort of 474 pHGG patients, with 258 (54%) and 216 (46%) patients positive and negative, respectively, for the H3K27M mutation. Overall, the presence of the mutation was independently and significantly associated with a worse prognosis (HR 3.630, p < 0.001). Overall survival was significantly shorter (by 2.300 years; p = 0.008) when the H3K27M mutation was present in pHGG. Meta-regression did not identify any study covariates of heterogeneous concern.

CONCLUSIONS

According to the current literature, pHGG patients positive for the H3K27M mutation are more than 3 times more susceptible to succumbing to this disease by more than 2 years, compared to patients negative for the mutation. More robust outcome data are required to improve our quantitative understanding of this pathological entity in order to assist in prioritizing clinical management. Future larger prospective studies are required to overcome inherent biases in the current literature to validate the quantitative findings of this study.

Full access

Weston Northam, Kristi Hildebrand, Scott Elton, and Carolyn Quinsey

Full access

Han Yan, Eric Toyota, Melanie Anderson, Taylor J. Abel, Elizabeth Donner, Suneil K. Kalia, James Drake, James T. Rutka, and George M. Ibrahim

OBJECTIVE

Drug-resistant epilepsy (DRE) presents a therapeutic challenge in children, necessitating the consideration of multiple treatment options. Although deep brain stimulation (DBS) has been studied in adults with DRE, little evidence is available to guide clinicians regarding the application of this potentially valuable tool in children. Here, the authors present the first systematic review aimed at understanding the safety and efficacy of DBS for DRE in pediatric populations, emphasizing patient selection, device placement and programming, and seizure outcomes.

METHODS

The systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and recommendations. Relevant articles were identified from 3 electronic databases (MEDLINE, Embase, and Cochrane CENTRAL) from their inception to November 17, 2017. Inclusion criteria of individual studies were 1) diagnosis of DRE; 2) treatment with DBS; 3) inclusion of at least 1 pediatric patient (age ≤ 18 years); and 4) patient-specific data. Exclusion criteria for the systematic review included 1) missing data for age, DBS target, or seizure freedom; 2) nonhuman subjects; and 3) editorials, abstracts, review articles, and dissertations.

RESULTS

This review identified 21 studies and 40 unique pediatric patients (ages 4–18 years) who received DBS treatment for epilepsy. There were 18 patients with electrodes placed in the bilateral or unilateral centromedian nucleus of the thalamus (CM) electrodes, 8 patients with bilateral anterior thalamic nucleus (ATN) electrodes, 5 patients with bilateral and unilateral hippocampal electrodes, 3 patients with bilateral subthalamic nucleus (STN) and 1 patient with unilateral STN electrodes, 2 patients with bilateral posteromedial hypothalamus electrodes, 2 patients with unilateral mammillothalamic tract electrodes, and 1 patient with caudal zona incerta electrode placement. Overall, 5 of the 40 (12.5%) patients had an International League Against Epilepsy class I (i.e., seizure-free) outcome, and 34 of the 40 (85%) patients had seizure reduction with DBS stimulation.

CONCLUSIONS

DBS is an alternative or adjuvant treatment for children with DRE. Prospective registries and future clinical trials are needed to identify the optimal DBS target, although favorable outcomes are reported with both CM and ATN in children.