Functional Network Development in Sagittal Craniosynostosis Treated With Whole Vault Cranioplasty.

Introduction: In this study, the authors seek to clarify the neurological changes before and after whole vault cranioplasty (WVC) in patients born with sagittal craniosynostosis.

Methods: A case control study design was performed that included thirty functional MRI scans, from 25 individual patients. Functional MRI and diffusion tension imaging data were analyzed with BioImageSuite (Yale University, USA).

Long-Term Neurocognitive Outcomes in Sagittal Synostosis: The Impact of Reoperation.

Introduction: Optimal age at surgery in nonsyndromic sagittal craniosynostosis continues to be debated. Previous reports suggest that earlier age at whole vault cranioplasty more frequently requires reoperation. It is unknown, however, whether reoperation affects neurocognitive outcome.

Exome sequencing implicates genetic disruption of prenatal neuro-gliogenesis in sporadic congenital hydrocephalus.

Congenital hydrocephalus (CH), characterized by enlarged brain ventricles, is considered a disease of excessive cerebrospinal fluid (CSF) accumulation and thereby treated with neurosurgical CSF diversion with high morbidity and failure rates. The poor neurodevelopmental outcomes and persistence of ventriculomegaly in some post-surgical patients highlight our limited knowledge of disease mechanisms. Through whole-exome sequencing of 381 patients (232 trios) with sporadic, neurosurgically treated CH, we found that damaging de novo mutations account for >17% of cases, with five different genes exhibiting a significant de novo mutation burden.

Understanding the Learning Disabilities Linked to Sagittal Craniosynostosis.

Objective: The purpose of this study is to investigate further findings that corroborate similarities between corrected sagittal craniosynostosis and attention deficit hyperactivity disorder (ADHD). The aim is to further characterize the neurocognitive deficits seen in adolescents with corrected craniosynostosis by comparing it to established learning deficits such as ADHD.

Methods: A total of 30 functional magnetic resonance imaging (fMRI) of 10 sagittal nonsyndromic craniosynostosis (sNSC), 10 ADHD-combined, and 10 control adolescents were studied.

De Novo Mutation in Genes Regulating Neural Stem Cell Fate in Human Congenital Hydrocephalus.

Congenital hydrocephalus (CH), featuring markedly enlarged brain ventricles, is thought to arise from failed cerebrospinal fluid (CSF) homeostasis and is treated with lifelong surgical CSF shunting with substantial morbidity. CH pathogenesis is poorly understood. Exome sequencing of 125 CH trios and 52 additional probands identified three genes with significant burden of rare damaging de novo or transmitted mutations: TRIM71 (p = 2.

Two locus inheritance of non-syndromic midline craniosynostosis via rare and common alleles.

Premature fusion of the cranial sutures (craniosynostosis), affecting 1 in 2000 newborns, is treated surgically in infancy to prevent adverse neurologic outcomes. To identify mutations contributing to common non-syndromic midline (sagittal and metopic) craniosynostosis, we performed exome sequencing of 132 parent-offspring trios and 59 additional probands. Thirteen probands (7%) had damaging de novo or rare transmitted mutations in , an inhibitor of BMP - induced osteoblast differentiation (p<10).

The effects of a state concussion law on the frequency of sport-related concussions as seen in two emergency departments.

Background: Connecticut (CT) passed its original sport-related concussion (SRC) law (PA 10-62) in 2010. The law requires that a health-care professional evaluate high school athletes with concussion symptoms. The purpose of this study was to evaluate two level 1 Trauma Center Emergency Department (ED) records for SRC before and after the Connecticut Public Act (CT PA) 10-62 to determine if the law had an effect on the presentation to the ED of SRCs.

Normalization of brain morphology after surgery in sagittal craniosynostosis.

OBJECT Nonsyndromic craniosynostosis (NSC) is associated with significant learning disability later in life. Surgical reconstruction is typically performed before 1 year of age to correct the cranial vault morphology and to allow for normalized brain growth with the goal of improving cognitive function. Yet, no studies have assessed to what extent normalized brain growth is actually achieved.

The impact of age at surgery on long-term neuropsychological outcomes in sagittal craniosynostosis.

Background: The relationship between surgical age and long-term neuropsychological outcomes in sagittal-suture craniosynostosis remains equivocal. Whole-vault cranioplasty and strip craniectomy are performed at various times in individuals with sagittal-suture craniosynostosis. This study used comprehensive neurological testing to examine the relationship between age at time of surgery and long-term neuropsychological function.

Thoracic epidural teratoma: case report and review of the literature.

Purpose: Spinal teratomas comprise a rare subset of spinal cord tumors, and here, we describe an even rarer childhood thoracic extradural-intracanalicular teratoma. The clinical presentation, management, and pathophysiology of these tumors are reviewed to promote recognition and guide treatment of these lesions.

Methods: We report the case of a 21-month-old boy who presented with marked spasticity, as well as failure to ambulate and meet motor milestones.

Intracranial infantile hemangiopericytoma.

Intracranial infantile hemangiopericytomas (HPCs) are exceedingly rare lesions. Only 11 cases have been previously reported in the literature. As such, little is known about the etiology, long-term prognosis, and optimal treatment paradigm.

Back to the basics: identifying positive youth development as the theoretical framework for a youth drug prevention program in rural Saskatchewan, Canada amidst a program evaluation.

Background: Despite endorsement by the Saskatchewan government to apply empirically-based approaches to youth drug prevention services in the province, programs are sometimes delivered prior to the establishment of evidence-informed goals and objectives. This paper shares the 'preptory' outcomes of our team's program evaluation of the Prince Albert Parkland Health Region Mental Health and Addiction Services' Outreach Worker Service (OWS) in eight rural, community schools three years following its implementation. Before our independent evaluation team could assess whether expectations of the OWS were being met, we had to assist with establishing its overarching program goals and objectives and 'at-risk' student population, alongside its alliance with an empirically-informed theoretical framework.

Decompressive hemicraniectomy for ischemic stroke in the pediatric population.

Adult patients with space-occupying hemispheric infarctions have a poor prognosis, with an associated fatality rate of 80%. Decompressive hemicraniectomy (DH) has been studied as a treatment option for patients with malignant cerebral infarction refractory to maximal medical therapy, with reasonable outcomes demonstrated in the adult population if the patient is decompressed within 48 h. However, there are no randomized controlled trials in the pediatric literature to make the same claims.

Intracranial compartment volume changes in sagittal craniosynostosis patients: influence of comprehensive cranioplasty.

Background: A retrospective analysis of intracranial compartment volume changes in children with sagittal craniosynostosis was performed to clarify the therapeutic objectives of corrective surgery.

Methods: Chart and computed tomographic review of 53 consecutive children with previously unoperated sagittal synostosis was performed, and preoperative and postoperative computed tomographic scans were examined and compared with 143 age- and gender-matched controls.

Results: Preoperative mean intracranial compartment volume and mean brain tissue volume of each subgroup were age dependent.

Primary myxopapillary ependymoma of the medulla: case report.

Objective: Myxopapillary ependymoma is a subclassification of ependymoma that is thought to be nearly exclusive to the conus medullaris or filum terminale. Primary intracerebral or brainstem myxopapillary ependymomas are rare.

Clinical Presentation: An 8-year-old child presented with a 5-month history of nausea and vomiting and a 1-week history of headache.

Primary, non-exophytic, optic nerve germ cell tumors.

Tumors of the optic chiasm are relatively uncommon and usually associated with phakomatoses such as neurofibromatosis. Even more rare is the presentation of a primary, non-exophytic, isolated optic chiasm germ cell tumor (GCT). These tumors have imaging characteristics nearly indistinguishable from optic chiasmatic gliomas (OCGs).

Longitudinal brain volume changes in preterm and term control subjects during late childhood and adolescence.

Objective: Although preterm very low birth weight infants have a high prevalence of neuroanatomical abnormalities when evaluated at term-equivalent age, patterns of brain growth in prematurely born infants during school age and adolescence remain largely unknown. Our goal was to test the hypothesis that preterm birth results in long-term dynamic changes in the developing brain.

Methods: We performed serial volumetric MRI studies at ages 8 and 12 years in 55 preterm infants born weighing 600 to 1250 g and 20 term control children who participated in the follow-up component of a prospective, randomized, placebo-controlled intraventricular hemorrhage prevention study.

Gastric bypass: a risk factor for neural tube defects? Case report.

Gastric bypass surgery has become a safe and acceptable surgical weight loss treatment for individuals who suffer from morbid obesity. Patients who undergo this procedure are subject to vitamin deficiencies due to an iatrogenic malabsorptive state. Folate, a vitamin known for its role in the prevention of neural tube defects (NTDs), can be part of the deficiency spectrum resulting from this procedure.

The misdiagnosis of craniosynostosis as deformational plagiocephaly.

Deformational plagiocephaly, unilateral coronal craniosynostosis, and lambdoid craniosynostosis all cause oblique deformities of the skull. They are separate entities with different etiologies resulting in clear differences of physical examination, radiologic findings, and clinical course. Treatment for each condition is markedly different.

Intracranial volume and cephalic index outcomes for total calvarial reconstruction among nonsyndromic sagittal synostosis patients.

Background: Controversy abounds as to how craniosynostosis affects intracranial volume and whether more extensive procedures achieve superior results. Intracranial volume and cephalic index were evaluated among nonsyndromic sagittal synostosis patients undergoing cranioplasty.

Methods: Twenty-four children with isolated nonsyndromic sagittal synostosis underwent a total calvarial reconstruction.

Cortical recruitment patterns in children born prematurely compared with control subjects during a passive listening functional magnetic resonance imaging task.

Objectives: To use functional magnetic resonance imaging (fMRI) to test the hypothesis that subjects who were born prematurely develop alternative systems for processing language.

Study Design: Subjects who were born prematurely (n = 14; 600-1250 g birthweight) without neonatal brain injury and 10 matched term control subjects were examined with a fMRI passive listening task of language, the Clinical Evaluation of Language Fundamentals (CELF) and portions of the Comprehensive Test of Phonological Processing (CTOPP). The fMRI task was evaluated for both phonologic and semantic processing.

A functional magnetic resonance imaging study of the long-term influences of early indomethacin exposure on language processing in the brains of prematurely born children.

Background: Previous studies have demonstrated that indomethacin lowers the incidence and decreases the severity of intraventricular hemorrhage, as well as improves the cognitive outcome, in prematurely born male infants.

Objective: The purpose of this work was to use functional magnetic resonance imaging to test the hypothesis that neonatal indomethacin treatment would differentially affect brain activation across genders in school-aged, prematurely born children during performance of a language task.

Methods: Forty-seven prematurely born children (600-1250-g birth weight) and 24 matched term control subjects were evaluated using a functional magnetic resonance imaging passive language task and neurodevelopmental assessments that included the Wechsler Intelligence Scale for Children-III and the Peabody Picture Vocabulary Test-Revised.