Reimagining the N-Of-1 Trial Within Pediatric Neuro-Oncology: A Shifting Paradigm.

The field of neuro-oncology has seen significant advances that have allowed the expansion of the therapeutic armamentarium. Nevertheless, overall outcomes have not improved significantly particularly for high-grade tumors. The relative rarity of these pathologies in the pediatric population limits the capacity to design large-scale, multicenter, randomized clinical trials.

Influence of focused ultrasound on locoregional drug delivery to the brain: Potential implications for brain tumor therapy.

Introduction: Efficient delivery of therapeutics across the blood-brain barrier (BBB) for the treatment of central nervous system (CNS) tumors is a major challenge to the development of safe and efficacious therapies. Locoregional drug delivery platforms offer an improved therapeutic index by achieving high drug concentrations in the target tissue with negligible systemic exposure. Intrathecal (intraventricular) [IT] and convection-enhanced delivery [CED] are two clinically relevant methods being employed for various CNS malignancies.

Innovations in the Diagnosis and Surgical Management of Low-Grade Gliomas.

Low-grade gliomas are a broad category of tumors that can manifest at different stages of life. As a group, their prognosis has historically been considered to be favorable, and surgery is a mainstay of treatment. Advances in the molecular characterization of individual lesions has led to newer classification systems, a better understanding of the biological behavior of different neoplasms, and the identification of previously unrecognized entities.

Weighing the Risk Profile of Cervical Spine MRI in Evaluating Pediatric Cervical Spine Injuries.

Objective: Screening for cervical spine injury after blunt trauma is common, but there remains varied practice patterns and clinical uncertainty regarding adequate radiographic evaluation. An oft-cited downside of MRI is the added risk compared to CT in the pediatric population; however, these specific risks have not yet been reported. This study examines the risks of cervical spine MRI in pediatric trauma patients in the context of what value MRI adds.

Acromegaly: Medical and Surgical Considerations.

Acromegaly results from excessive secretion of insulinlike growth factor-1 and growth hormone, which most commonly occurs because of pituitary somatotrophinoma. Diagnostic features of acromegaly include elevated insulinlike growth factor-1 and growth hormone; lesion on brain MRI; and clinically dysmorphic features, such as soft tissue swelling, jaw prognathism, and acral overgrowth. Transsphenoidal resection is the primary therapy for individuals with acromegaly, even in the cases where gross total resection is not possible because of parasellar extension and cavernous sinus involvement.

Transseptal interforniceal endoscopic removal of superiorly recessed colloid cysts.

Objective: Transforaminal endoscopic colloid cyst resection is well described. However, some anatomical colloid cyst variants may warrant a modified approach. Rarely, colloid cysts separate the forniceal columns and grow superiorly within the leaflets of the septum pellucidum.

Closed-loop vagal nerve stimulation for intractable epilepsy: A single-center experience.

Purpose: A new class of heart-rate sensing, closed-loop vagal nerve stimulator (VNS) devices for refractory epilepsy may improve seizure control by using pre-ictal autonomic changes as an indicator for stimulation. We compared our experience with closed- versus open-loop stimulator implantation at a single institution.

Methods: We conducted a retrospective chart review of consecutive VNS implantations performed from 2004 to 2018.

Arachnoid cysts: using prenatal imaging and need for pediatric neurosurgical intervention to better understand their natural history and prognosis.

Objectives: Intracranial arachnoid cysts are commonly characterized as congenital. Evidence to support a congenital origin is scant and documented evolution during infancy also calls into question the genesis of these lesions. To improve our understanding of the natural history and the clinical significance of arachnoid cysts on prenatal ultrasound, we conducted a study to describe the fate of these cysts after initial diagnosis.

Utility of invasive electroencephalography in children 3 years old and younger with refractory epilepsy.

Objective: Early surgical intervention for pediatric refractory epilepsy is increasingly advocated as surgery has become safer and data have demonstrated improved outcomes with early seizure control. There is concern that the risks associated with staged invasive electroencephalography (EEG) in very young children outweigh the potential benefits. Here, the authors present a cohort of children with refractory epilepsy who were referred for invasive monitoring, and they evaluate the role and safety of staged invasive EEG in those 3 years old and younger.

Syringomyelia Resolution Following Chiari Surgery: A Novel Scale for Communication and Research.

Background: The pathophysiological connection between Chiari malformation and syringomyelia is accepted. Debate remains, however, how can we best define changes in syringomyelia following surgery.

Objective: To introduce a grading system focusing on syrinx reduction based on routinely and reproducible radiological information, and provide a suggestion of the application of this scale for prediction of patient's prognoses.

The slope of the learning curve in 600 consecutive endoscopic transsphenoidal pituitary surgeries.

Background: Endonasal endoscopic transsphenoidal surgery (EETS) for pituitary adenoma has become a mainstay of treatment over the last two decades and it is generally accepted that once this learning curve is achieved, a plateau is reached with little incremental improvement.

Objective: The objective of this study was to assess the slope of the learning curve over a long period of time for a variety of outcomes measures.

Methods: We examined outcomes and complications in a consecutive series of 600 EETS for pituitary adenoma grouped into quartiles based on date of surgery.

Ventrolateral Tonsillar Position Defines Novel Chiari 0.5 Classification.

Background: Cervicomedullary compression in young children has been described in the context of Chiari type 1 malformation, with symptoms associated with the extent of tonsillar herniation below McRae line. Historically, Chiari type 1 malformation has been defined by tonsillar herniation of at least 5 mm. However, in certain populations, including very young children, Chiari symptoms may be present without this finding.

How long is the tail end of the learning curve? Results from 1000 consecutive endoscopic endonasal skull base cases following the initial 200 cases.

Objective: Endoscopic endonasal approaches (EEAs) to the skull base have evolved over the last 20 years to become an essential component of a comprehensive skull base practice. Many case series show a learning curve from the earliest cases, in which the authors were inexperienced or were not using advanced closure techniques. It is generally accepted that once this learning curve is achieved, a plateau is reached with little incremental improvement.

Incidence and risk factors associated with reoperation for sellar hematoma following endoscopic transsphenoidal pituitary surgery.

Objective: Postoperative sellar hematoma is an uncommon complication of endonasal endoscopic transsphenoidal surgery (EETS) for pituitary adenoma that often requires emergency surgical evacuation. Sellar hematomas can cause mass effect and compress parasellar structures, leading to clinically significant symptoms such as visual impairment and severe headache. The objective of this study was to determine the incidence and risk factors associated with reoperation for postoperative hematoma after EETS for pituitary adenoma.

A Dual Approach for the Management of Complex Craniovertebral Junction Abnormalities: Endoscopic Endonasal Odontoidectomy and Posterior Decompression with Fusion.

Background: Ventral brainstem compression secondary to complex craniovertebral junction abnormality is an infrequent cause of neurologic deterioration in pediatric patients. However, in cases of symptomatic, irreducible ventral compression, 360° decompression of the brainstem supported by posterior stabilization may provide the best opportunity for improvement in symptoms. More recently, the endoscopic endonasal corridor has been proposed as an alternative method of odontoidectomy associated with less morbidity.

Magnetic resonance-guided focused ultrasound for ablation of mesial temporal epilepsy circuits: modeling and theoretical feasibility of a novel noninvasive approach.

Objective: The authors tested the feasibility of magnetic resonance-guided focused ultrasound (MRgFUS) ablation of mesial temporal lobe epilepsy (MTLE) seizure circuits. Up to one-third of patients with mesial temporal sclerosis (MTS) suffer from medically refractory epilepsy requiring surgery. Because current options such as open resection, laser ablation, and Gamma Knife radiosurgery pose potential risks, such as infection, hemorrhage, and ionizing radiation, and because they often produce visual or neuropsychological deficits, the authors developed a noninvasive MRgFUS ablation strategy for mesial temporal disconnection to mitigate these risks.

Endoscopic removal of recurrent colloid cysts.

Objective: Removal of colloid cysts of the third ventricle using a purely endoscopic method has been established as a safe and advantageous technique. It is hypothesized that endoscopic removal in recurrent cases might pose more technical challenges and result in less success. The objective of this study was to assess the feasibility and outcomes of using a purely endoscopic approach for the management of recurrent colloid cysts compared to primary cysts.

Single incision endoscope-assisted surgery for sagittal craniosynostosis.

Objective: The objective of this study is to present the novel technique and associated results of a single-incision endoscope-assisted procedure for the treatment of sagittal craniosynostosis.

Methods: We retrospectively reviewed the charts of infants who underwent single-incision endoscope-assisted sagittal craniectomy for craniosynostosis at our institution. Demographic data collected included patient age, blood loss, operative time, pre- and post-operative hemoglobin, pre- and post-operative cephalic index (CI), and hospital length of stay.

Normative ranges of anthropometric cranial indices and metopic suture closure during infancy.

OBJECTIVE Subjective evaluations typically guide craniosynostosis repair. This study provides normative values of anthropometric cranial indices that are clinically useful for the evaluation of multiple types of craniosynostosis and introduces 2 new indices that are useful in the evaluation and management of metopic and bicoronal synostosis. The authors hypothesize that normative values of the new indices as well as for established measures like the cephalic index can be drawn from the evaluation of CT scans of normal individuals.