Ultrasound-guided transuterine coil embolization of a Vein of Galen malformation.

Vein of Galen malformations (VOGMs) are rare and complex congenital vascular anomalies that can lead to severe morbidity and mortality. Management predominantly focuses on postnatal endovascular interventions. However, these may not be feasible for fetuses with hemodynamic instability and high-output cardiac failure and may fail to prevent irreversible brain damage induced by prolonged compression by the venous varix, hemodynamic alterations and resultant potential ischemic injury.

Triptolide and its prodrug Minnelide target high-risk MYC-amplified medulloblastoma in preclinical models.

Most children with medulloblastoma (MB) achieve remission, but some face very aggressive metastatic tumors. Their dismal outcome highlights the critical need to advance therapeutic approaches that benefit such high-risk patients. Minnelide, a clinically relevant analog of the natural product triptolide, has oncostatic activity in both preclinical and early clinical settings.

Evaluating the potential role of determinants of health on encephalocele patient outcomes - a combined retrospective study and systematic review.

Introduction: To evaluate the outcomes and demographics of encephalocele patients who were born and received treatment in our neonatal ICU and conduct a PRISMA literature review.

Methods: An Institutional Review Board (IRB)-approved retrospective cohort study was undertaken to investigate the results of treating encephalocele patients at Jackson Memorial Hospital (JMH) from 1998 to 2022. The study focused on assessing outcomes and the impact of maternal socioeconomic factors, such as religion, age, and education, along with the timing of diagnosis, in connection with a systematic review.

Using intent to get ahead of pediatric cranial firearm injuries.

Objective: Pediatric firearm injuries (PFI) are a public health crisis. Little is known about how injury intent may influence the outcome of pediatric cranial firearm injuries (PCFI). The current study sought to compare demographics and outcomes of PCFI based on intent of injury.

Prognostic and Diagnostic Utility of Serum Biomarkers in Pediatric Traumatic Brain Injury.

Traumatic brain injury (TBI) remains a major cause of morbidity and death among the pediatric population. Timely diagnosis, however, remains a complex task because of the lack of standardized methods that permit its accurate identification. The aim of this study was to determine whether serum levels of brain injury biomarkers can be used as a diagnostic and prognostic tool in this pathology.

Treating Delinquent and Feebleminded Juveniles at the Beloit Industrial School for Girls in Early Twentieth-Century Kansas.

This study explores the troubling and unintended consequences of public health efforts to address the problem of juvenile delinquency and feeblemindedness. Health care professionals, superintendents, and other authority figures equated undesirable juvenile behaviors such as keeping "bad company" or "falling in with the wrong crowd," truancy, and petty theft with poor breeding, low intelligence, and inheritable criminal tendencies. This article interrogates historical documentation culled from the Kansas State Historical Society (KSHS) and focuses on a few specific cases to reveal the ways a patriarchal political and medical state system both protected and alienated young woman accused of a myriad of behavior issues including delinquency, incorrigibility, and feeblemindedness.

Assessment of neuromonitoring use and postoperative readmission rates in pediatric Chiari I malformation with syrinx.

Introduction: While operative intervention for Chiari malformation type I (CMI) with syringomyelia is well established, there is limited data on outcomes of intraoperative neuromonitoring (IONM). This study sought to explore differences in procedural characteristics and their effects on postoperative readmission rates.

Methods: The Nationwide Readmission Database was queried from 2010 to 2014 for patients ≤ 18 years of age with CMI and syringomyelia who underwent cranial decompression or spinal decompression.

Clinical course of pediatric gunshot wounds involving the spine and spinal cord: the Miami experience.

Objective: Civilian gunshot wounds (GSWs) involving the skeletal spine and spinal cord in pediatric patients are fortunately rare. Nevertheless, their presentation mandates judicious evaluation, and their clinical outcomes remain poorly defined. Thus, the authors aimed to characterize the clinical course of this traumatic presentation in the pediatric population based on their institutional experience.

A Druggable UHRF1/DNMT1/GLI Complex Regulates Sonic Hedgehog-Dependent Tumor Growth.

Unlabelled: Dysregulation of Sonic hedgehog (SHH) signaling drives the growth of distinct cancer subtypes, including medulloblastoma (MB). Such cancers have been treated in the clinic with a number of clinically relevant SHH inhibitors, the majority of which target the upstream SHH regulator, Smoothened (SMO). Despite considerable efficacy, many of these patients develop resistance to these drugs, primarily due to mutations in SMO.

Noncanonical activation of GLI signaling in SOX2 cells drives medulloblastoma relapse.

SRY (sex determining region Y)-box 2 (SOX2)-labeled cells play key roles in chemoresistance and tumor relapse; thus, it is critical to elucidate the mechanisms propagating them. Single-cell transcriptomic analyses of the most common malignant pediatric brain tumor, medulloblastoma (MB), revealed the existence of astrocytic cells expressing sonic hedgehog (SHH) signaling biomarkers. Treatment with vismodegib, an SHH inhibitor that acts on Smoothened (Smo), led to increases in astrocyte-like cells.

Intraarterial delivery of bevacizumab and cetuximab utilizing blood-brain barrier disruption in children with high-grade glioma and diffuse intrinsic pontine glioma: results of a phase I trial.

Objective: Delivery of drugs intraarterially to brain tumors has been demonstrated in adults. In this study, the authors initiated a phase I trial of superselective intraarterial cerebral infusion (SIACI) of bevacizumab and cetuximab in pediatric patients with refractory high-grade glioma (diffuse intrinsic pontine glioma [DIPG] and glioblastoma) to determine the safety and efficacy in this population.

Methods: SIACI was used to deliver mannitol (12.

Large sphenoid mucocele presenting with cranial neuropathies in a 10-year-old boy: case report and literature review.

Introduction: Mucoceles in the sphenoid sinus are rare, making up 1-3% of all paranasal sinus mucoceles. Sphenoid sinus mucoceles among pediatric patients are uncommon and have a range of presentations due to their proximity to other structures, in rare cases causing oculomotor and visual disturbances through expansion and mass effect.

Case Report: We present a case of a large expansile sphenoid sinus mucocele causing cranial nerve III and VI palsies in a 10-year-old boy.

Intracranial arachnoid cysts in an infant: A technical note on the innovative use of navigation and flexible endoscopy for cyst fenestration.

Background: Intracranial arachnoid cysts (ACs) are a cerebral spinal fluid (CSF) collection within the meninges. They typically arise during embryologic development. Some are stable overtime with little consequence, but large or growing cysts may require surgical intervention.

Colloid cysts of the third ventricle in children.

Objective: The rarity of colloid cysts in children makes it difficult to characterize this entity and offer meaningful advice on treatment. Infrequent case reports exist, but to date there has been no age-specific assessment. The purpose of this study was to define any differences between children and adults who are evaluated and treated for colloid cysts of the third ventricle.

Myelomeningocele-associated hydrocephalus: nationwide analysis and systematic review.

Objective: Myelomeningocele (MMC), the most severe form of spina bifida, is characterized by protrusion of the meninges and spinal cord through a defect in the vertebral arches. The management and prevention of MMC-associated hydrocephalus has evolved since its initial introduction with regard to treatment of MMC defect, MMC-associated hydrocephalus treatment modality, and timing of hydrocephalus treatment.

Methods: The Nationwide Inpatient Sample (NIS) database from the years 1998-2014 was reviewed and neonates with spina bifida and hydrocephalus status were identified.

Increased sensitivity to traumatic axonal injury on postconcussion diffusion tensor imaging scans in National Football League players by using premorbid baseline scans.

Objective: Statistical challenges exist when using diffusion tensor imaging (DTI) to assess traumatic axonal injury (TAI) in individual concussed athletes. The authors examined active professional American football players over a 6-year time period to study potential TAI after concussion and assess optimal methods to analyze DTI at the individual level.

Methods: Active American professional football players recruited prospectively were assessed with DTI, conventional MRI, and standard clinical workup.

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series.

Objective: Few studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.

Methods: A retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017.

Hypoxia-enhanced Blood-Brain Barrier Chip recapitulates human barrier function and shuttling of drugs and antibodies.

The high selectivity of the human blood-brain barrier (BBB) restricts delivery of many pharmaceuticals and therapeutic antibodies to the central nervous system. Here, we describe an in vitro microfluidic organ-on-a-chip BBB model lined by induced pluripotent stem cell-derived human brain microvascular endothelium interfaced with primary human brain astrocytes and pericytes that recapitulates the high level of barrier function of the in vivo human BBB for at least one week in culture. The endothelium expresses high levels of tight junction proteins and functional efflux pumps, and it displays selective transcytosis of peptides and antibodies previously observed in vivo.

EGFR amplification and classical subtype are associated with a poor response to bevacizumab in recurrent glioblastoma.

Purpose: The highly vascular malignant brain tumor glioblastoma (GBM) appears to be an ideal target for anti-angiogenic therapy; however, clinical trials to date suggest the VEGF antibody bevacizumab affects only progression-free survival. Here we analyze a group of patients with GBM who received bevacizumab treatment at recurrence and are stratified according to tumor molecular and genomic profile (TCGA classification), with the goal of identifying molecular predictors of the response to bevacizumab.

Methods: We performed a retrospective review of patients with a diagnosis of glioblastoma who were treated with bevacizumab in the recurrent setting at our hospital, from 2006 to 2014.

A CK1α Activator Penetrates the Brain and Shows Efficacy Against Drug-resistant Metastatic Medulloblastoma.

Purpose: Although most children with medulloblastoma are cured of their disease, Sonic Hedgehog (SHH) subgroup medulloblastoma driven by mutations is essentially lethal. Casein kinase 1α (CK1α) phosphorylates and destabilizes GLI transcription factors, thereby inhibiting the key effectors of SHH signaling. We therefore tested a second-generation CK1α activator against -mutant, -amplified medulloblastoma.

Superselective intraarterial cerebral infusion of cetuximab after osmotic blood/brain barrier disruption for recurrent malignant glioma: phase I study.

Objective To establish a maximum tolerated dose of superselective intraarterial cerebral infusion (SIACI) of Cetuximab after osmotic disruption of the blood-brain barrier (BBB) with mannitol, and examine safety of the procedure in patients with recurrent malignant glioma. Methods A total of 15 patients with recurrent malignant glioma were included in the current study. The starting dose of Cetuximab was 100 mg/m(2) and dose escalation was done to 250 mg/m(2).

Pediatric Suprasellar Tumors.

The various childhood suprasellar tumors, while pathologically distinct, present similar clinical and surgical challenges as a result of their common anatomic location. These lesions are in close proximity to or may invade the optic nerve and chiasm, pituitary gland and infundibulum, hypothalamus, and third ventricle, leading to presenting features including visual field loss, impairment in visual acuity, endocrine dysfunction, and hydrocephalus. Though many suprasellar lesions are relatively benign in pathology, treatment may be complicated by high surgical morbidity resulting from damage to the hypothalamic-pituitary axis.

Sex, Age, Anatomic Location, and Extent of Resection Influence Outcomes in Children With High-grade Glioma.

Background: Survival duration and prognostic factors in adult high-grade glioma have been comprehensively analyzed, but less is known about factors contributing to overall survival (OS) and progression-free survival (PFS) in pediatric patients.

Objective: To identify these factors in the pediatric population.

Methods: We retrospectively reviewed institutional databases evaluating all patients ≤21 years with high-grade glioma treated between 1988 and 2010.