Concurrent myelomeningocele and sagittal craniosynostosis: illustrative case.

Background: Myelomeningocele and sagittal craniosynostosis are 2 neurosurgical pathologies with complications such as increased intracranial pressure (ICP) and hydrocephalus. While the 2 defects commonly occur independently, their simultaneous occurrence is exceptionally rare.

Observations: The authors report the case of a newborn male diagnosed with a simultaneous myelomeningocele and sagittal craniosynostosis.

Infantile traumatic pericallosal aneurysm: illustrative case.

Background: Traumatic aneurysms are a rare sequela of nonaccidental head trauma in infants. The rate of nonaccidental trauma (NAT) in the pediatric population is increasing; therefore, traumatic aneurysms are an important consideration in the evaluation of pediatric patients with abusive head trauma.

Observations: A 24-day-old infant with no significant past medical or birth history presented with twitching and poor oral intake for 1 day.

Exoscope Efficacy and Feasibility in Pediatric Spinal Neurosurgery: A Single-Institution Cohort Case Series.

Background: The exoscope has emerged as an efficacious microscope in adult spinal neurosurgery providing improved operative field visibility and surgeon ergonomics. However, outcome data and feasibility are underrepresented in the pediatric literature. We present the largest case series aimed at assessing operative and clinical outcomes in pediatric patients undergoing various exoscope-assisted spinal surgeries.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Symptoms.

Background: Chiari I malformation (CIM) is characterized by descent of the cerebellar tonsils through the foramen magnum, potentially causing symptoms from compression or obstruction of the flow of cerebrospinal fluid. Diagnosis and treatment of CIM is varied, and guidelines produced through systematic review may be helpful for clinicians.

Objective: To perform a systematic review of the medical literature to answer specific questions on the diagnosis and treatment of CIM.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Diagnosis.

Background: Chiari I malformation (CIM) is characterized by descent of the cerebellar tonsils through the foramen magnum, potentially causing symptoms from compression or obstruction of the flow of cerebrospinal fluid. Diagnosis and treatment of CIM is varied, and guidelines produced through systematic review may be helpful for clinicians.

Objective: To perform a systematic review of the medical literature to answer specific questions on the diagnosis and treatment of CIM.

Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Patients With Chiari Malformation: Surgical Interventions.

Background: Chiari malformation type I (CIM) diagnoses have increased in recent years. Controversy regarding the best operative management prompted a review of the literature to offer guidance on surgical interventions.

Objective: To assess the literature to determine (1) whether posterior fossa decompression or posterior fossa decompression with duraplasty is more effective in preoperative symptom resolution; (2) whether there is benefit from cerebellar tonsillar resection/reduction; (3) the role of intraoperative neuromonitoring; (4) in patients with a syrinx, how long should a syrinx be observed for improvement before additional surgery is performed; and 5) what is the optimal duration of follow-up care after preoperative symptom resolution.

A Multicenter Study Investigating the Surgeon Experience with a Robotic-Assisted Exoscope as Part of the Neurosurgical Armamentarium.

Background: Improvement of visualization tools in neurosurgery such as the exoscope has raised the question of how this technology compares to the conventional microscope for surgeon ergonomics, discomfort, and patient outcomes. Exoscopes have the advantage of greater optical zoom, resolution, and illumination at a lower light intensity. Heads-up display for both the primary surgeon and other assistants permits neutral positioning of the surgeons while placing the camera in more angled positions.

Interactions between programmable shunt valves and magnetically controlled growing rods for scoliosis.

Objective: Although the advent of magnetic growing rod technology for scoliosis has provided a means to bypass multiple hardware lengthening operations, it is important to be aware that many of these same patients have a codiagnosis of hydrocephalus with magnet-sensitive programmable ventricular shunts. As the magnetic distraction of scoliosis rods has not previously been described to affect the shunt valve setting, the authors conducted an investigation to characterize the interaction between the two devices.

Methods: In this ex vivo study, the authors carried out 360 encounters between four different shunt valve types at varying distances from the magnetic external remote control (ERC) used to distract the growing rods.

Preconditioning with INC280 and LDK378 drugs sensitizes MGMT-unmethylated glioblastoma to temozolomide: Pre-clinical assessment.

Temozolomide (TMZ) therapy is the standard of care for patients with glioblastoma (GBM). Clinical studies have shown that elevated levels of DNA repair protein O (6)-methylguanine-DNA methyltransferase (MGMT) or deficiency/defect of DNA mismatch repair (MMR) genes is associated with TMZ resistance in some, but not all, GBM tumors. Another reason for GBM treatment failure is signal redundancy due to coactivation of several functionally linked receptor tyrosine kinases (RTKs), including anaplastic lymphoma kinase (ALK) and c-Met (hepatocyte growth factor receptor).

Effect of Dovitinib (TKI258), a Multi-Target Angiokinase Inhibitor on Aggressive Meningioma Cells.

Meningiomas represent ∼30% of primary central nervous system (CNS) tumors. Although advances in surgery and radiotherapy have significantly improved survival, there remains an important subset of patients whose tumors have more aggressive behavior and are refractory to conventional therapy. Recent advances in molecular genetics and epigenetics suggest that this aggressive behavior may be due to the deletion of the DNA repair and tumor suppressor gene, neurofibromatosis Type 2 (NF2) mutation on chromosome 22q12, and genetic abnormalities in multiple RTKs including FGFRs.

Chiari I malformation in children with transverse myelitis.

Purpose: Transverse myelitis (TM) is an acute inflammatory spinal cord injury. Asymptomatic Chiari I malformation (CMI) management is highly controversial, particularly when associated with a spinal syrinx. Here, we assess the occurrence of CMI in the pediatric TM population and management outcomes.

Re-Examining the Need for Tissue Diagnosis in Pediatric Diffuse Intrinsic Pontine Gliomas: A Review.

Diffuse intrinsic pontine glioma (DIPG) is a malignant brain tumor of childhood that carries an extremely poor prognosis. There are ~200-300 new cases diagnosed each year, [1, 2] and little progress has been made in changing the prognosis and outcome of the tumor since it was first documented in the literature in 1926 [3]. The median overall survival is 8-11 months [4], with an overall survival rate of 30% at 1 year, and less than 10% at 2 years [4].

Enzyme replacement therapy for congenital hypophosphatasia allows for surgical treatment of related complex craniosynostosis: a case series.

Hypophosphatasia (HPP) is a rare inherited disorder of bone metabolism that results in the loss of function of the gene coding for tissue-nonspecific alkaline phosphatase (TNSALP). Patients with HPP have defective bone mineralization as well as craniosynostosis that can be seen in the infantile and childhood forms of this disease. Traditionally, HPP has had a poor prognosis, with few children surviving to exhibit the phenotype of clinical craniosynostosis that requires surgical intervention.

High long-term symptomatic recurrence rates after Chiari-1 decompression without dural opening: a single center experience.

Objective: We evaluated long-term outcomes of posterior fossa decompression (PFD) without duroplasty in consecutive patients with newly diagnosed Chiari-1 malformation.

Methods: We searched the institutional database for all adult patients who underwent Chiari decompression between 1995 and 2007. PFD was offered to all consecutive patients with Chairi-1 malformation during this time period.

Occipital condyle to cervical spine fixation in the pediatric population.

Fixation at the craniovertebral junction (CVJ) is necessary in a variety of pediatric clinical scenarios. Traditionally an occipital bone to cervical fusion is preformed, which requires a large amount of hardware to be placed on the occiput of a child. If a patient has previously undergone a posterior fossa decompression or requires a decompression at the time of the fusion procedure, it can be difficult to anchor a plate to the occipital bone.