Socioeconomic and demographic factors in the diagnosis and treatment of Chiari malformation type I and syringomyelia.

Objective: The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM).

Methods: The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes.

Gadolinium is not necessary for surveillance MR imaging in children with chiasmatic-hypothalamic low-grade glioma.

Background: Patients with chiasmatic-hypothalamic low-grade glioma (CHLGG) have frequent MRIs with gadolinium-based contrast agents (GBCA) for disease monitoring. Cumulative gadolinium deposition in the brains of children is a potential concern. The purpose of this study is to evaluate whether MRI with GBCA is necessary for determining radiographic tumor progression in children with CHLGG.

Comparison of multimodal surgical and radiation treatment methods for pediatric craniopharyngioma: long-term analysis of progression-free survival and morbidity.

Objective: The authors compared survival and multiple comorbidities in children diagnosed with craniopharyngioma who underwent gross-total resection (GTR) versus subtotal resection (STR) with radiation therapy (RT), either intensity-modulated radiation therapy (IMRT) or proton beam therapy (PBT). The authors hypothesized that there are differences between multimodal treatment methods with respect to morbidity and progression-free survival (PFS).

Methods: The medical records of children diagnosed with craniopharyngioma and treated surgically between February 1997 and December 2018 at Texas Children's Hospital were reviewed.

Occipital-Cervical Fusion and Ventral Decompression in the Surgical Management of Chiari-1 Malformation and Syringomyelia: Analysis of Data From the Park-Reeves Syringomyelia Research Consortium.

Background: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology.

Objective: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD.

Methods: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD.

A Novel Sand Dollar and Staves Technique for Unilateral Lambdoid Craniosynostosis.

Background: Unilateral lambdoid synostosis (ULS) is the rarest form of craniosynostosis. Due to the associated cranio-caudal shift seen in ULS, surgical correction is technically challenging from a morphological standpoint. Herein, the authors present a novel "Sand-Dollar and Staves" technique for the repair of ULS.

Superior Intellectual Outcomes After Proton Radiotherapy Compared With Photon Radiotherapy for Pediatric Medulloblastoma.

Purpose: Proton radiotherapy (PRT) may lessen the neuropsychological risk traditionally associated with cranial radiotherapy for the treatment of pediatric brain tumors by reducing the dose to normal tissue compared with that of photon radiotherapy (XRT). We examined the change in intellectual scores over time in patients with pediatric medulloblastoma treated with craniospinal PRT versus XRT.

Methods: Intelligence test scores were obtained for a sample of pediatric patients treated between 2007 and 2018 on the same medulloblastoma protocols that differed only in radiotherapy modality (PRT XRT).

Chicken or the Egg: A Case Report of Endoscopic-Assisted Treatment of Concomitant Sagittal Craniosynostosis With Calcified Cephalohematoma.

Nonsyndromic craniosynostosis results in premature fusion of cranial sutures and is rarely found in the context of other craniofacial abnormalities. Here the authors present the case of a 3-month-old male infant with a rare presentation of sagittal craniosynostosis and concomitant calcified cephalohematoma repaired by endoscopic-assisted sagittal strip craniectomy with good cosmetic and functional outcomes. The authors discuss the advantages of endoscopic repair of craniosynostosis when found in the presence of a cephalohematoma and the need for further research to investigate a possible causal relationship between these 2 pathologies.

Nonsyndromic Craniosynostosis.

Nonsyndromic craniosynostosis is significantly more common than syndromic craniosynostosis, affecting the sagittal, coronal, metopic, and lambdoid sutures in decreasing order of frequency. Nonsyndromic craniosynostosis is most frequently associated with only 1 fused suture, creating a predictable head shape. Repair of craniosynostosis is recommended to avoid potential neurodevelopmental delay.

Increased risk of pseudoprogression among pediatric low-grade glioma patients treated with proton versus photon radiotherapy.

Background: Pseudoprogression (PsP) is a recognized phenomenon after radiotherapy (RT) for high-grade glioma but is poorly characterized for low-grade glioma (LGG). We sought to characterize PsP for pediatric LGG patients treated with RT, with particular focus on the role of RT modality using photon-based intensity-modulated RT (IMRT) or proton beam therapy (PBT).

Methods: Serial MRI scans from 83 pediatric LGG patients managed at 2 institutions between 1998 and 2017 were evaluated.

Hypothyroidism after craniospinal irradiation with proton or photon therapy in patients with medulloblastoma.

Background: Craniospinal irradiation (CSI) often results in endocrine deficiencies in children with medulloblastoma due to irradiation of the hypothalamic-pituitary axis (HPA) or the thyroid gland. CSI with Proton radiation therapy (PRT) has the potential to decrease the risk of hypothyroidism by reduction in radiation dose to these organs. This study compares the risk for hypothyroidism in patients with medulloblastoma treated with Photon radiation therapy (XRT) or PRT.

Indirect revascularization with the dural inversion technique for pediatric moyamoya disease: 20-year experience.

OBJECTIVENumerous surgical procedures facilitate revascularization of the ischemic brain in patients with moyamoya disease. Dural inversion is a technique in which flaps of dura mater centered around the middle meningeal artery are inverted, encouraging the formation of a rich collateral blood supply. This procedure has been used in combination with encephaloduroarteriosynangiosis for more than 20 years at the authors' institution for the treatment of pediatric moyamoya disease.

CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH-associated neurodegeneration and mass lesions.

Background: Central nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH.

Methods: Cerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions.

Progression-free survival of children with localized ependymoma treated with intensity-modulated radiation therapy or proton-beam radiation therapy.

Background: The treatment for childhood intracranial ependymoma includes maximal surgical resection followed by involved-field radiotherapy, commonly in the form of intensity-modulated radiation therapy (IMRT). Proton-beam radiation therapy (PRT) is used at some centers in an effort to decrease long-term toxicity. Although protons have the theoretical advantage of a minimal exit dose to the surrounding uninvolved brain tissue, it is unknown whether they have the same efficacy as photons in preventing local recurrence.

Imaging Changes in Pediatric Intracranial Ependymoma Patients Treated With Proton Beam Radiation Therapy Compared to Intensity Modulated Radiation Therapy.

Purpose: The clinical significance of magnetic resonance imaging (MRI) changes after radiation therapy (RT) in children with ependymoma is not well defined. We compared imaging changes following proton beam radiation therapy (PBRT) to those after photon-based intensity modulated RT (IMRT).

Methods And Materials: Seventy-two patients with nonmetastatic intracranial ependymoma who received postoperative RT (37 PBRT, 35 IMRT) were analyzed retrospectively.

Long-term disease control and toxicity outcomes following surgery and intensity modulated radiation therapy (IMRT) in pediatric craniopharyngioma.

Purpose: To report long-term progression-free survival (PFS) and late-toxicity outcomes in pediatric craniopharyngioma patients treated with IMRT.

Patients And Methods: Twenty-four children were treated with IMRT to a median dose of 50.4Gy (range, 49.

Silencing BMI1 eliminates tumor formation of pediatric glioma CD133+ cells not by affecting known targets but by down-regulating a novel set of core genes.

Clinical outcome of children with malignant glioma remains dismal. Here, we examined the role of over-expressed BMI1, a regulator of stem cell self-renewal, in sustaining tumor formation in pediatric glioma stem cells. Our investigation revealed BMI1 over-expression in 29 of 54 (53.

Therapeutic implications of CD1d expression and tumor-infiltrating macrophages in pediatric medulloblastomas.

Immunobiology of medulloblastoma (MB), the most common malignant brain tumor in children, is poorly understood. Although tumor cells in some MBs were recently shown to express CD1d and be susceptible to Vα24-invariant natural killer T (NKT)-cell cytotoxicity, the clinical relevance of CD1d expression in MB patients remains unknown. We investigated the expression of CD1d in pediatric MBs and correlated with molecular and clinical characteristics.

Novel somatic and germline mutations in intracranial germ cell tumours.

Intracranial germ cell tumours (IGCTs) are a group of rare heterogeneous brain tumours that are clinically and histologically similar to the more common gonadal GCTs. IGCTs show great variation in their geographical and gender distribution, histological composition and treatment outcomes. The incidence of IGCTs is historically five- to eightfold greater in Japan and other East Asian countries than in Western countries, with peak incidence near the time of puberty.

Long-term outcome of centrally located low-grade glioma in children.

Background: Optimal management of children with centrally located low-grade glioma (LGG) is unclear. Initial interventions in most children are chemotherapy in younger and radiation therapy (RT) in older children. A better understanding of the inherent risk factors along with the effects of interventions on long-term outcome can lead to reassessment of the current approaches to minimize long-term morbidity.

Implications of tumor location on subtypes of medulloblastoma.

Background: Medulloblastoma (MB) comprises of four molecular subtypes, Sonic hedgehog (SHH), Wingless (WNT), Groups 3 and 4. WNT-subtype MBs were found to arise from midline of the brainstem occupying the fourth ventricle while SHH-subtype occupied the cerebellar hemisphere in a small subset of patients.

Procedure: We tested this hypothesis in a large cohort of pediatric MBs comprising of all four molecular subtypes.

Management of ruptured dissecting intracranial aneurysms in infants: report of four cases and review of the literature.

Background: Intracranial cerebral aneurysms in the pediatric population are infrequent, and those occurring in infants less than 1 year old are extremely rare. Of intracranial aneurysms in children, dissecting aneurysms are the most common type seen. While spontaneous dissecting aneurysms usually present with ischemia, hemorrhage can also occur.

Development of secondary unilateral coronal suture synostosis with a sagittal suture synostosis in a nonsyndromic patient.

Multiple-suture synostosis is typically associated with syndromic craniosynostosis but has been occasionally reported in large series of nonsyndromic children. The diagnosis of multiple fused sutures usually occurs at the same time, but rarely has the chronological development of a secondary suture synostosis been noted. The development of secondary bicoronal suture synostosis requiring surgical intervention has only been reported, to date, after surgical intervention and is hypothesized to arise from a disruption of inhibitory factors from the dura.