Purpose: Although conventional surgery is presently used to treat seizures of temporolimbic and neocortical origin, deep‐seated lesions are often associated with morbidity. Stereotactic radiosurgery ...is a noninvasive procedure that effectively treats patients with vascular malformations and brain tumors, but its efficacy for epileptogenic foci is limited, especially in children.
Methods: Between 1995 and 1999, four candidates who had medically uncontrolled seizures and localized seizure foci were selected for stereotactic radiosurgery, with a mean age of 9.75 years at the time of surgery (range, 4–17 years). Seizure foci were identified on the basis of ictal and interictal video‐EEG. Magnetic resonance (MR) images were obtained before and after surgery. Ictal single‐photon emission computed tomography (SPECT) was performed by using stabilized hexamethyl‐propyleneamine oxime (HMPAO; 300 microcuries/kg) with early injection after electrographic ictal onset. The clinical features of the patients are given. All radiosurgical procedures were performed with the gamma knife unit with the Leksell stereotactic frame, stereotactic MRI imaging, and the Gamma Plan workstation. Seizure outcome was scored according to Engel's classification.
Results: Two patients had hypothalamic hamartoma (HH), and two had neocortical epilepsy. At mean follow‐up of 39.2 months (range, 26–69 months), two patients were seizure free, one with a HH and one with a suggestive developmental tumor in the insular cortex by MRI findings. The other patient with HH had 90% reduction of seizures. One patient with a widespread seizure focus that involved the motor strip was unimproved. The two patients with HH also exhibited markedly improved neurobehavioral status after surgery. There were no significant complications of radiosurgical therapy.
Conclusions: Our findings suggest that gamma knife surgery is a potentially valuable treatment modality for children with medically intractable epilepsy due to a well‐localized seizure focus that is difficult to excise by conventional techniques or for whom they are deemed unsuitable. More widespread application in childhood epilepsy should be investigated in larger series.
IMPORTANCE: Mild traumatic brain injury (mTBI), or concussion, in children is a rapidly growing public health concern because epidemiologic data indicate a marked increase in the number of emergency ...department visits for mTBI over the past decade. However, no evidence-based clinical guidelines have been developed to date for diagnosing and managing pediatric mTBI in the United States. OBJECTIVE: To provide a guideline based on a previous systematic review of the literature to obtain and assess evidence toward developing clinical recommendations for health care professionals related to the diagnosis, prognosis, and management/treatment of pediatric mTBI. EVIDENCE REVIEW: The Centers for Disease Control and Prevention (CDC) National Center for Injury Prevention and Control Board of Scientific Counselors, a federal advisory committee, established the Pediatric Mild Traumatic Brain Injury Guideline Workgroup. The workgroup drafted recommendations based on the evidence that was obtained and assessed within the systematic review, as well as related evidence, scientific principles, and expert inference. This information includes selected studies published since the evidence review was conducted that were deemed by the workgroup to be relevant to the recommendations. The dates of the initial literature search were January 1, 1990, to November 30, 2012, and the dates of the updated literature search were December 1, 2012, to July 31, 2015. FINDINGS: The CDC guideline includes 19 sets of recommendations on the diagnosis, prognosis, and management/treatment of pediatric mTBI that were assigned a level of obligation (ie, must, should, or may) based on confidence in the evidence. Recommendations address imaging, symptom scales, cognitive testing, and standardized assessment for diagnosis; history and risk factor assessment, monitoring, and counseling for prognosis; and patient/family education, rest, support, return to school, and symptom management for treatment. CONCLUSIONS AND RELEVANCE: This guideline identifies the best practices for mTBI based on the current evidence; updates should be made as the body of evidence grows. In addition to the development of the guideline, CDC has created user-friendly guideline implementation materials that are concise and actionable. Evaluation of the guideline and implementation materials is crucial in understanding the influence of the recommendations.
Appropriate differentiation between epidermoid-dermoid and arachnoid cysts is important for patient management. MRI has had limitations in differentiating these two types of cysts, especially in the ...region of the spinal cord.
To evaluate the role of diffusion-weighted (DW) MRI for differentiation between spinal arachnoid and epidermoid-dermoid cysts.
Four children underwent conventional and DW MRI. Turbo spin-echo imaging was used to obtain DW images.
On conventional MRI, low T1-W and high T2-W signal was present in all lesions. DW imaging showed restricted diffusion in two patients with epidermoid-dermoid cysts and no water restriction in two patients with arachnoid cysts. Three of four children had extramedullary lesions and one patient had an intramedullary lesion. Three children had surgery, two with epidermoid-dermoid cysts and one with arachnoid cyst. Diagnosis was confirmed by histopathology. One child with a thoracic arachnoid cyst had stable clinical imaging findings during a follow-up of 21 months.
On conventional MRI, epidermoid-dermoid and arachnoid cysts have similar signal characteristics. Initial experience with spine DW imaging shows promise in differentiating epidermoid-dermoid cysts from arachnoid cysts. Preoperative differentiation is important because it changes management and surgical approach.
Vertical Orbital Dystopia (VOD) results in significant facial asymmetry, psychological distress, and poor quality of life in affected patients. The traditional approach (TA) for surgical correction ...has entailed a standard frontal craniotomy along with circumferential orbital osteotomy, vertical translocation of the orbit, and bone grafting to the lower maxilla. Caution has been expressed regarding its invasive transcranial nature. In this report, we describe the limited approach (LA) for simplified surgical correction of VOD, which obviates the need for a standard frontal craniotomy.
A 45-year retrospective review was conducted of all patients that underwent surgical correction of VOD, as performed by a single surgeon. Demographic details, procedural characteristics, and complications were compared between patients who were corrected by the TA and those who were corrected by the LA. Complications were defined as CSF leak, infection of the frontal bone, permanent diplopia, permanent ptosis, sudden-onset vision loss, persistent asymmetry, and surgical revision.
Forty patients met inclusion criteria for correction of true VOD, of which 18 underwent the TA and 22 underwent the LA. Mean length of hospital stay was 5.3±2.3 days and 4.0±1.5 days for the TA and LA cohorts, respectively. Mean follow-up time was 4.9±7.5 years for the TA and 2.6±3.3 years for the LA. The only reported complications were persistent asymmetry in 2 patients in the TA cohort with one patient requiring surgical revision due to undercorrection, while the LA cohort exhibited no postoperative asymmetry or need for surgical revision.
Both the TA and LA are effective for surgical correction of VOD. The limited craniotomy of the LA reduces exposure of intracranial structures and adequately achieves postoperative symmetry.
IMPORTANCE: In recent years, there has been an exponential increase in the research guiding pediatric mild traumatic brain injury (mTBI) clinical management, in large part because of heightened ...concerns about the consequences of mTBI, also known as concussion, in children. The CDC National Center for Injury Prevention and Control’s (NCIPC) Board of Scientific Counselors (BSC), a federal advisory committee, established the Pediatric Mild TBI Guideline workgroup to complete this systematic review summarizing the first 25 years of literature in this field of study. OBJECTIVE: To conduct a systematic review of the pediatric mTBI literature to serve as the foundation for an evidence-based guideline with clinical recommendations associated with the diagnosis and management of pediatric mTBI. EVIDENCE REVIEW: Using a modified Delphi process, the authors selected 6 clinical questions on diagnosis, prognosis, and management or treatment of pediatric mTBI. Two consecutive searches were conducted on PubMed, Embase, ERIC, CINAHL, and SportDiscus. The first included the dates January 1, 1990, to November 30, 2012, and an updated search included December 1, 2012, to July 31, 2015. The initial search was completed from December 2012 to January 2013; the updated search, from July 2015 to August 2015. Two authors worked in pairs to abstract study characteristics independently for each article selected for inclusion. A third author adjudicated disagreements. The risk of bias in each study was determined using the American Academy of Neurology Classification of Evidence Scheme. Conclusion statements were developed regarding the evidence within each clinical question, and a level of confidence in the evidence was assigned to each conclusion using a modified GRADE methodology. Data analysis was completed from October 2014 to May 2015 for the initial search and from November 2015 to April 2016 for the updated search. FINDINGS: Validated tools are available to assist clinicians in the diagnosis and management of pediatric mTBI. A significant body of research exists to identify features that are associated with more serious TBI-associated intracranial injury, delayed recovery from mTBI, and long-term sequelae. However, high-quality studies of treatments meant to improve mTBI outcomes are currently lacking. CONCLUSIONS AND RELEVANCE: This systematic review was used to develop an evidence-based clinical guideline for the diagnosis and management of pediatric mTBI. While an increasing amount of research provides clinically useful information, this systematic review identified key gaps in diagnosis, prognosis, and management.
The objective of this in vitro tissue study is to investigate the feasibility of using optical spectroscopy to differentiate pediatric neoplastic and epileptogenic brain from normal brain. Specimens ...are collected from 17 patients with brain tumors, and from 26 patients with intractable epilepsy during surgical resection of epileptogenic cerebral cortex. Fluorescence spectra are measured at excitations of 337, 360, and 440 nm; diffuse reflectance spectra are measured between 400 and 900 nm from each specimen. Pathological analysis is performed to classify abnormalities in brain specimens, and its findings are correlated with spectral data. Statistically significant differences (p<0.01) are found for both raw and normalized diffuse reflectance and fluorescence spectra between 1. neoplastic brain and normal gray matter, 2. epileptogenic brain and normal gray matter, and 3. neoplastic brain and normal white matter. However, no distinct spectral features are identified that effectively separate epileptogenic brain from normal white matter. The outcomes of the study suggest that certain unique compositional and structural characteristics of pediatric neoplastic and epileptogenic brain can be detected using optical spectroscopy in vitro.
Abstract
BACKGROUND
Pleomorphic xanthoastrocytoma (PXA) and anaplastic pleomorphic xanthoastrocytoma (APXA) are two types of rare astrocytomas in pediatrics. There is limited literature reporting ...their outcomes.
METHODS
A retrospective analysis of pediatric patients with diagnosis of PXA and APXA treated at Nicklaus Children’s Hospital was conducted using descriptive measures.
RESULTS
A chart review of patients with brain tumors from 2001 to 2019 revealed that 13 patients (median age 6 years, range 2–17 years) were diagnosed with non-metastatic PXA (n=12) and APXA (n=1). Six patients (46%) were male. Clinical presentation included seizures (n=8), headaches (n=2) cranial nerve palsies (n=2). Diagnostic imaging showed tumor in the temporal lobe (n=5), parietal lobe (n=2), temporoparietal lobe (n=2), frontal lobe (n=1), occipital lobe (n=1), others (n=2). BRAF V600E mutation were identified in three of five analyzed tumors including PXA (n=4) and APXA (n=1). Surgical intervention consisted of gross total resection (n=8) and near/subtotal resection (n=5). Ten patients (76%) required a second surgery (median 2; range 1–4) due to local recurrence. The median time to recurrence was 3 years (range 6 months - 7 years). Three patients (23%) had malignant transformation of PXA, which occurred three to eight years from the initial surgery; one of them also had extensive spinal metastasis. Focal radiation was given to six patients (46%) due to multiple recurrence (n=2), malignant transformation (n=3), APXA (n=1). At a median follow up of 8 years (range 3 to 20 years), eight patients remain alive, three patients had lost to follow up, two patients died from progressive disease. Long-term sequelae varied as follows; seizures (n=5), motor weakness (n=4), cranial nerve palsies (n=2), and learning disability (n=1).
CONCLUSION
PXA is associated with high local recurrence rate and uncommonly malignant transformation to APXA in pediatrics, which requires close follow-up due to unpredictable biological behavior of these tumors.
Intracranial mixed vascular malformations (MVMs) are defined as any combination of a developmental venous anomaly (DVA), cerebral cavernous malformation (CCM), capillary telangiectasia (CTG), or ...arteriovenous malformation (AVM) within a single, contiguous lesion. However, most MVMs described in the literature contain only 2 pathologically discrete malformations; juxtaposition of 3 or more abnormalities in a single lesion remains exceedingly rare. We present the case of a 19-month-old female with new onset focal seizures and a 4-cm right basal ganglia lesion initially believed to be an embryonal neoplasm. She subsequently underwent gross total resection (GTR) of the lesion via a transsylvian-transinsular approach. Intraoperatively, the lesion appeared to be heterogenous and highly vascular, with areas of purplish-gray friable tissue. Pathology confirmed the lesion to be a MVM containing a CCM, CTG, and a DVA. This appears to be the first reported case of such a lesion confirmed on pathology in the literature.
Resection and disconnection surgeries for epilepsy in the pediatric demographic (patients ≤ 18 years of age) are two separate, definitive intervention options in medically refractory cases. Questions ...remain regarding the role of surgery when seizures persist after an initial incomplete surgery. The aim of this study was to review the contemporary literature and summarize the metadata on the outcomes of repeat surgery in this specific demographic.
Searches of seven electronic databases from inception to July 2022 were conducted using PRISMA guidelines. Articles were screened using prespecified criteria. Metadata from the articles were abstracted and pooled by random-effects meta-analysis of proportions.
Eleven studies describing 12 cohorts satisfied all criteria, reporting outcomes of 170 pediatric patients with epilepsy who underwent repeat resection or disconnection surgery. Of these patients, 55% were male, and across all studies, median ages at initial and repeat surgeries were 7.2 and 9.4 years, respectively. The median follow-up duration after repeat surgery was 47.7 months. The most commonly reported etiology for epilepsy was cortical dysplasia. Overall, the estimated incidence of complete seizure freedom (Engel class I) following repeat surgery was 48% (95% CI 40%-56%, p value for heterogeneity = 0.93), and the estimated incidence of postoperative complications following repeat surgery was 25% (95% CI 12%-39%, p = 0.04). There were six cohorts each that described outcomes for repeat resection and repeat disconnection surgeries. There was no statistical difference between these two subgroups with respect to estimated incidence of complete seizure freedom (p value for interaction = 0.92), but postoperative complications were statistically more common following repeat resection (p ≤ 0.01).
For both resection and disconnection surgeries, repeat epilepsy surgery in children is likely to confer complete seizure freedom in approximately half of the patients who experience unsuccessful initial incomplete epilepsy surgery. More data are needed to elucidate the impact on efficacy based on surgical approach selection. Judicious discussion and planning between the patient, family, and a multidisciplinary team of epilepsy specialists is recommended to optimize expectations and outcomes in this setting.
Focused ultrasound (FUS) is a noninvasive therapeutic technology with multiple pediatric clinical applications. The ability of focused ultrasound to target tissues deep in the body without exposing ...children to the morbidities associated with conventional surgery, interventional procedures, or radiation offers significant advantages. In 2021, there are 10 clinical pediatric focused ultrasound studies evaluating various musculoskeletal, oncologic, neurologic, and vascular diseases of which 8 are actively recruiting and 2 are completed. Pediatric musculoskeletal applications of FUS include treatment of osteoid osteoma and bone metastases using thermal ablation and high-intensity FUS. Pediatric oncologic applications of FUS include treatment of soft tissue tumors including desmoid tumors, malignant sarcomas, and neuroblastoma with high-intensity FUS ablation alone, or in combination with targeted chemotherapy delivery. Pediatric neurologic applications include treatment of benign tumors such as hypothalamic hamartomas with thermal ablation and malignant diffuse intrinsic pontine glioma with low-intensity FUS for blood brain barrier opening and targeted drug delivery. Additionally, low-intensity FUS can be used to treat seizures. Pediatric vascular applications of FUS include treatment of arteriovenous malformations and twin-twin transfusion syndrome using ablation and vascular occlusion. FUS treatment appears safe and efficacious in pediatric populations across many subspecialties. Although there are 7 Food and Drug Administration-approved indications for adult applications of FUS, the first Food and Drug Administration approval for pediatric patients with osteoid osteoma was obtained in 2020. This review summarizes the preclinical and clinical research on focused ultrasound of potential benefit to pediatric populations.
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