Objective:
To compare postoperative temporal expansion in patients treated with fronto-orbital advancement or endoscopy-assisted craniectomy with cranial orthotic therapy.
Design:
This is a ...retrospective, multicenter cohort study of patients with unilateral coronal craniosynostosis (UCS).
Setting:
Computed tomographic (CT) scans were drawn from UCS patients treated at Boston Children’s Hospital or St Louis Children’s Hospital.
Patients:
The study included 56 patients with UCS after fronto-orbital advancement (n = 32) or endoscopic repair (n = 24) and 10 age-matched controls.
Intervention:
Fronto-orbital advancement entails a craniotomy of the frontal bone and superior orbital rim followed by reshaping and forward advancement. Endoscopic repair is the release of the synostotic suture and guidance of further growth of the cranium using a molding orthotic.
Main Outcome Measures:
Measures included posterior temporal width, anterior temporal width, orbital width, and anterior cranial fossa area taken preoperatively and 1 year postoperatively. Linear regression was performed to assess 1 year postoperative improvement in symmetry; covariates included preoperative symmetry and type of surgery.
Results:
Both treatments showed improvement in orbital width and anterior cranial fossa area symmetry 1 year postoperatively (P < .001), but no significant improvement in posterior or anterior temporal width symmetry. Linear regression revealed no difference between the 2 procedures in any of the 4 measurements (.096 ≤ P ≤ .898).
Conclusions:
Fronto-orbital advancement and endoscopic repair show equivalent outcomes 1 year postoperatively in all 3 width measurements and anterior cranial fossa area. Neither procedure produced significant improvement in temporal width.
The authors report the case of a 14-year-old male with a subependymal giant cell astrocytoma (SEGA) that occurred in the absence of tuberous sclerosis complex (TSC). The patient presented with ...progressive headache and the sudden onset of nausea and vomiting. Neuroimaging revealed an enhancing left ventricular mass located in the region of the foramen of Monro with significant mass effect and midline shift. The lesion had radiographic characteristics of SEGA; however, the diagnosis remained unclear given the absence of clinical features of TSC. The patient underwent gross-total resection of the tumor with resolution of his symptoms. Although tumor histology was consistent with SEGA, genetic analysis of both germline and tumor DNA revealed no TSC1/2 mutations. Similarly, a comprehensive clinical evaluation failed to reveal any clinical features characteristic of TSC. Few cases of SEGA without clinical or genetic evidence of TSC have been reported. The histogenesis, genetics, and clinical approach to this rare lesion are briefly reviewed.
BACKGROUND:Outcomes research on Chiari malformation type 1 (CM-1) is impeded by a reliance on small, single-center cohorts.
OBJECTIVE:To study the complications and resource use associated with adult ...CM-1 surgery using administrative data.
METHODS:We used a recently validated International Classification of Diseases, Ninth Revision, Clinical Modification code algorithm to retrospectively study adult CM-1 surgeries from 2004 to 2010 in California, Florida, and New York using State Inpatient Databases. Outcomes included complications and resource use within 30 and 90 days of treatment. We used multivariable logistic regression to identify risk factors for morbidity and negative binomial models to determine risk-adjusted costs.
RESULTS:We identified 1947 CM-1 operations. Surgical complications were more common than medical complications at both 30 days (14.3% vs 4.4%) and 90 days (18.7% vs 5.0%) postoperatively. Certain comorbidities were associated with increased morbidity; for example, hydrocephalus increased the risk for surgical (odds ratio OR = 4.51) and medical (OR = 3.98) complications. Medical but not surgical complications were also more common in older patients (OR = 5.57 for oldest vs youngest age category) and male patients (OR = 3.19). Risk-adjusted hospital costs were $22530 at 30 days and $24852 at 90 days postoperatively. Risk-adjusted 90-day costs were more than twice as high for patients experiencing surgical ($46264) or medical ($65679) complications than for patients without complications ($18880).
CONCLUSION:Complications after CM-1 surgery are common, and surgical complications are more frequent than medical complications. Certain comorbidities and demographic characteristics are associated with increased risk for complications. Beyond harming patients, complications are also associated with substantially higher hospital costs. These results may help guide patient management and inform decision making for patients considering surgery.
ABBREVIATIONS:CM-1, Chiari malformation type 1ICD-9-CM, International Classification of Diseases, Ninth Revision, Clinical ModificationOR, odds ratioSID, State Inpatient Database
OBJECTIVE Electrical status epilepticus of sleep (ESES) is a rare electrographic pattern associated with global regression, which is often poorly responsive to traditional epilepsy treatments and can ...have a devastating and permanent neurocognitive outcome. The authors analyzed clinical, electroencephalographic, and neuropsychological outcomes in 9 patients with refractory ESES treated with functional hemispherotomy to illustrate the wide clinical spectrum associated with the disease and explore the role of hemispherotomy in its treatment. METHODS During the period between 2003 and 2015, 80 patients underwent hemispherotomy at the authors' institution. Video electroencephalography (EEG) reports were reviewed for ESES or continuous spikes and waves during sleep (CSWS). Patients with preoperative ESES (> 85% slow-wave sleep occupied by spike waves), a unilateral structural lesion amenable to surgery, and more than 6 months of follow-up data were included in the analysis. Clinical data, EEG recordings, neuropsychological testing, and parental and clinician reports were retrospectively reviewed. RESULTS Nine patients were eligible for study inclusion. Age at seizure onset ranged from birth to 4.2 years (mean 1.9 years), age at ESES diagnosis ranged from 3.5 to 8.8 years (mean 6.0 years), and age at hemispherotomy ranged from 3.7 to 11.5 years (mean 6.8 years). All patients had drug-resistant epilepsy. The duration of epilepsy prior to hemispherotomy ranged from 2.7 to 8.9 years (mean ± SD, 5.0 ± 2.2 years). Engel Class I seizure outcome was observed in all 9 children, with a mean follow-up of 3.0 years (range 0.5-6.1 years). Hemispherotomy terminated ESES in 6 of 6 patients with available postoperative sleep EEG. All children had preoperative neuropsychological impairments. Developmental regression was halted postoperatively, but none of the children returned to their original pre-ESES baseline. Four children demonstrated academic gains, 2 of whom transitioned to mainstream classes. CONCLUSIONS Children with drug-resistant ESES and a unilateral structural lesion should be evaluated for hemispherotomy as they may experience the cessation of seizures, termination of ESES, and improvement in neuropsychological status.
The field of epilepsy surgery has seen tremendous growth in recent years. Innovative new devices have driven much of this growth, but some has been driven by revisions of existing products. Devices ...have also helped to rejuvenate existing procedures, as in the case of robotic assistance for electrode placement for stereo-electroencephalography, and these devices have brought significant attention along with their introduction. Other devices, such as responsive neurostimulators or laser interstitial thermal therapy systems, have introduced novel treatment modalities and broadened the surgical indications. Collectively, these advances are rapidly changing much of the landscape in the world of pediatric neurosurgery for medically refractory epilepsy. The foundations for indications for neurosurgical intervention are well supported in strong research data, which has also been expanded in recent years. In this article, the authors review advances in the neurosurgical treatment of pediatric epilepsy, beginning with trials that have repeatedly demonstrated the value of neurosurgical procedures for medically refractory epilepsy and following with several recent advances that are largely focused on less-invasive intervention.
ABBREVIATIONS AED = antiepileptic drug; ANT = anterior nucleus of the thalamus; BOLD = blood oxygen level dependent; CCEP = cortico-cortical evoked potential; DBS = deep brain stimulation; ECoG = electrocorticography; ERSET = Early Randomized Surgical Epilepsy Trial; FCD = focal cortical dysplasia; HH = hypothalamic hamartoma; LITT = laser interstitial thermal therapy; RCT = randomized controlled trial; r-fMRI = resting-state functional MRI; RNS = responsive neurostimulation; SEEG = stereo-electroencephalography; VNS = vagus nerve stimulation.
Precision medicine models for personalizing achieving sustained behavior change are largely outside of current clinical practice. Yet, changing self-regulatory behaviors is fundamental to the ...self-management of complex lifestyle-related chronic conditions such as depression and obesity - two top contributors to the global burden of disease and disability. To optimize treatments and address these burdens, behavior change and self-regulation must be better understood in relation to their neurobiological underpinnings. Here, we present the conceptual framework and protocol for a novel study, “Engaging self-regulation targets to understand the mechanisms of behavior change and improve mood and weight outcomes (ENGAGE)”. The ENGAGE study integrates neuroscience with behavioral science to better understand the self-regulation related mechanisms of behavior change for improving mood and weight outcomes among adults with comorbid depression and obesity. We collect assays of three self-regulation targets (emotion, cognition, and self-reflection) in multiple settings: neuroimaging and behavioral lab-based measures, virtual reality, and passive smartphone sampling. By connecting human neuroscience and behavioral science in this manner within the ENGAGE study, we develop a prototype for elucidating the underlying self-regulation mechanisms of behavior change outcomes and their application in optimizing intervention strategies for multiple chronic diseases.
•Uses a novel approach integrating neuroscience with behavioral medicine.•Includes self-regulation assays across lab, virtual reality, and natural settings.•Utilizes a brain-based experimental medicine approach to behavioral intervention.•Aims to identify self-regulation profiles to tailor intervention strategies.•Identifies a new taxonomy based on emotion, cognition, and self-reflection domains.
Introduction:
This study compares anthropometric outcomes of 2 sagittal synostosis repair techniques: spring-assisted surgery and endoscope-assisted craniectomy with molding helmet therapy.
Methods:
...Patients undergoing spring-assisted surgery (n = 27) or endoscope-assisted craniectomy with helmet therapy (n = 40) at separate institutions were retrospectively reviewed. Pre- and 1-year postoperative computed tomography (CT) or laser scans were analyzed for traditional cranial index (CI), adjusted cranial index (aCI), and cranial vault volume (CVV). Nine patient-matched scans were analyzed for measurement consistency.
Results:
The spring-assisted group was older at both time points (P < .050) and spring-assisted group CVV was larger preoperatively and postoperatively (P < .01). However, the change in CVV did not differ between the groups (P = .210). There was no difference in preoperative CI (helmet vs spring: 70.1 vs 71.2, P = .368) between the groups. Postoperatively, helmet group CI (77.0 vs 74.3, P = .008) was greater. The helmet group also demonstrated a greater increase in CI (6.9 vs 3.1, P < .001). The proportion of patients achieving CI of 75 or greater was not significantly different between the groups (helmet vs spring: CI, 65% vs 52%, P = .370). There was no detectable bias in CI between matched CT and laser scans. Differences were identified between scan types in aCI and CVV measurements; subsequent analyses used corrected CVV and aCI measures for laser scan measures.
Conclusions:
Both techniques had equivalent proportions of patients achieving normal CI, comparable effects on cranial volume, and similar operative characteristics. The study suggests that there may be greater improvement in CI in the helmet group. However, further research should be performed.
Glioblastoma in children: a single-institution experience Perkins, Stephanie M; Rubin, Joshua B; Leonard, Jeffrey R ...
International journal of radiation oncology, biology, physics,
07/2011, Volume:
80, Issue:
4
Journal Article
Peer reviewed
Current treatment recommendations for pediatric glioblastoma include surgery, chemotherapy, and radiation therapy. However, even with this multispecialty approach, overall survival remains poor. To ...assess outcome and evaluate treatment-related prognostic factors, we retrospectively reviewed the experience at our institution.
Twenty-four glioblastoma patients under the age of 21 were treated with radiation therapy with curative intent at Washington University, St. Louis, from 1970 to 2008. Patients underwent gross total resection, subtotal resection or biopsy alone. Fourteen (58%) of the patients received chemotherapy. All patients received radiation therapy. Radiation consisted of whole-brain radiation therapy in 7 (29%) patients with a median dose of 50.4 Gy. Seventeen (71%) patients received three-dimensional conformal radiation therapy with a median dose of 54 Gy.
Median follow-up was 12.5 months from diagnosis. One and 2-year overall survival rates were 57% and 32%, respectively. Median overall survival was 13.5 months. There were no differences in overall survival based on patients' age, race, gender, tumor location, radiation volume, radiation dose, or the use of chemotherapy. There was a significant improvement in overall survival for patients in whom gross total resection was achieved (p = 0.023). Three patients were alive 5 years after gross total resection, and 2 patients were alive at 10 and 24 years after diagnosis.
Survival for children with glioblastoma remains poor. Data from this and other studies demonstrate the importance of achieving a gross total resection. Continued investigation into new treatment options is needed in an attempt to improve outcome for these patients.
To identify factors, including the use of intraoperative magnetic resonance imaging (iMRI), impacting overall survival (OS) and progression-free survival (PFS) after resections of newly diagnosed ...intracranial grade II ependymomas performed across 4 different institutions.
Analyses of a multicenter mixed retrospective/prospective database assessed the impact of patient, treatment, and tumor characteristics on OS and PFS. iMRI workflow and logistics were also outlined.
Forty-three patients were identified (mean age 25.4 years, mean follow-up 52.8 months). The mean OS was 52.8 ± 44.7 months. Univariate analyses failed to identify prognostic factors associated with OS, likely due to relatively shorter follow-up time for this less aggressive glioma subtype. The mean PFS was 43.7 ± 39.8 months. Multivariate analyses demonstrated that gross-total resection was associated with prolonged PFS compared to both subtotal resection (STR) (P = 0.005) and near-total resection (P = 0.01). Infratentorial location was associated with improved PFS compared to supratentorial location (P = 0.04). Log-rank analyses of Kaplan–Meier survival curves showed that increasing extent of resection (EOR) led to improved OS specifically for supratentorial tumors (P = 0.02) and improved PFS for all tumors (P < 0.001). Thirty cases (69.8%) utilized iMRI, of which 12 (27.9%) involved additional resection after iMRI. Of these, 8/12 (66.7%) resulted in gross-total resection, while 2/12 (16.7%) were near-total resection and 2/12 (16.7%) were subtotal resection. iMRI was not an independent prognosticator of PFS (P = 0.72).
Greater EOR and infratentorial location were associated with increased PFS for grade II ependymomas. Greater EOR was associated with longer OS only for supratentorial tumors. A longer follow-up is needed to establish prognostic factors for this cohort, including use of iMRI.
Endoscopic strip craniectomy for metopic craniosynostosis relies on rapid growth and postoperative helmeting for correction. Endoscopic repair is generally performed before patients reach 4 months of ...age, and outcomes in older patients have yet to be quantified. Here, the authors examined a cohort of patients treated with endoscopic repair before or after 4 months of age to determine aesthetic outcomes of delayed repairs.
Data from eligible patients were retrospectively assessed and aggregated in a dedicated metopic synostosis database. Inclusion criteria were radiographically confirmed metopic synostosis and endoscopic treatment. Patients were dichotomized into two groups: those younger than 4 months and those 4 months or older at the time of repair. The frontal width and interfrontal divergence angle (IFDA) were measured on reconstructed CT images. These measurements, alongside operative time, estimated blood loss, and transfusion rates, were compared between groups using the Student t-test or chi-square test.
The study population comprised 28 patients treated before 4 months of age and 8 patients treated at 4-6 months of age. Patient sex and perioperative complications did not differ by age group. Older age at repair was not significantly associated with 1-year postoperative IFDA (140° ± 4.2° vs 142° ± 5.0°, p = 0.28) or frontal width (84 ± 5.2 vs 83 ± 4.4 mm, p = 0.47).
One-year postoperative IFDA and frontal width do not differ significantly between patients treated before and after 4 months of age. Further study with longer follow-up is necessary to confirm the longevity of these results at skeletal maturity.
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