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Introduction: Spinal astroblastoma is a rare highly malignant tumor that mainly affects children. We review the few cases described in the literature and highlight the challenges of managing this neoplasm by illustrating a case recently treated at our institutions. To our knowledge, this is the first published case of EWSR1-BEND2 fused spinal astroblastoma with long-term follow-up. Case Presentation: An 8-year-old girl was transferred from her home country to Switzerland for treatment of a recurrent intramedullary tumor of the cervical spine extending from C2-C7. The tumor was primarily diagnosed as an ependymoma of the spinal cord. Prior to her transfer to our department, the patient had undergone subtotal resection of the lesion, radiation therapy, multiple chemotherapy regimens, and biopsy of the recurrent tumor. Clinically, the patient presented with tetraparesis and had recently experienced worsening upper extremity weakness with complete loss of hand function. We performed a near total resection of the recurrent tumor. Ultra-fast Nanopore seq® based DNA methylome profiling allowed confirmation of the molecular diagnosis of a high-grade neuroepithelial tumor (HGNET-MN1) consistent with astroblastoma in less than 2 h, with subsequent molecular workup revealing a EWSR1-BEND2 fusion. After surgery, the patient gradually regained function in her hands. She was sent to a specialized pediatric rehabilitation center, and while the tumor was being followed radiologically with no adjuvant treatment planned, the patient presented with a relapse of the tumor in only 3 months. Given the acute worsening of radiating pain and sudden respiratory failure, a cervical decompression was performed. MRI of the cervical spine showed infiltration of the lower aspects of the brainstem. The patient was offered palliative comfort care. Conclusion: Spinal astroblastoma is a rare and highly aggressive tumor affecting children and young adults with a high recurrence rate and thus far not well-defined prognosis. The molecular signature of astroblastoma needs to be further characterized to establish a treatment-relevant classification and to allow a better prognostication. Currently, gross-total resection combined with radiotherapy remains the mainstay of treatment for spinal astroblastoma. [ABSTRACT FROM AUTHOR]

Positional plagiocephaly is a deformational cranial flattening frequently treated in pediatric neurosurgical practice. Positional maneuvers and orthotic helmet therapy are preferred therapeutic options for moderate-to-severe forms. Treatment response seems to be age-dependent. Nevertheless, predictive data are vague, and cost-efficiency might be a limiting factor for treatment. The purpose of this study was to investigate the early predictive value of sonographic parameters on the efficacy of orthotic helmet therapy through the assessment of changes in skull shape and correlation of the parameters with caliper cephalometry values and with age. A consecutive cohort of 49 patients < 10 months of age, undergoing orthotic helmet therapy for positional plagiocephaly, was recruited prospectively. The authors routinely assessed the patency of the lambdoid sutures by ultrasound and the following additional skull parameters were measured: suture width, adjacent full bone thickness, adjacent cortical bone thickness and occipital angle. Caliper cephalometric values, as well as demographic and clinical data were collected. Retrospective data analysis showed an inverse relation between both cortical and full skull bone thickness and early treatment efficacy, defined by a reduction in the occipital angle. The improvement of sonographic parameters correlated with the development of cranial caliper cephalometry values. In conclusion, the sonographic assessment of skull bone thickness is a safe and cost-effective tool to predict the early efficacy of orthotic helmet therapy in positional plagiocephaly and might, therefore, help the clinician to foresee the potential evolution of the deformity. [ABSTRACT FROM AUTHOR]

Objective: Transitional care in pediatric neurosurgery is challenging for patients and their parents. The specific needs of neurologically affected patients and the unique characteristics of the pathologies affecting pediatric neurosurgical patients compared with adults make a comprehensive, well-organized transition process essential for patient well-being and ensuring continuity of care. Little is known about patients' preferences and opinions on this topic. This study aimed to assess the patients' and parents' expectations and perceptions of the transition process., Methods: The authors retrospectively identified patients aged 16 to 30 years who underwent surgery in their pediatric neurosurgical department. The patients were divided into two groups: those about to transition and those who had already transitioned. Transition models were identified within the latter group. Parents of eligible patients were contacted for a telephone survey, and the patients themselves were included when possible. A modified version of the established Got Transition questionnaire from the National Alliance to Advance Adolescent Health was used., Results: Thirty-four patients were included, and 44 telephone surveys were conducted with the patients and their parents. Three transition models were applied, with 7 patients (41.2%) transitioned using the continued caregiver model, 9 patients (52.9%) using the shared caregiver model, and 1 patient (5.9%) using the specialized clinic model. Patient and parent satisfaction was highest among the patients transitioned using the continued caregiver and specialized clinic models., Conclusions: Neurosurgical conditions in children differ significantly from those in adults, creating unique transitional care challenges. The continuing caregiver model has been shown to provide continuity of care and high patient and parent satisfaction. However, there are differences in the perspectives of parents and patients regarding transitional care, with parents typically expressing greater concern and need for detailed information. Implementing a well-structured and individualized transition process is essential to reduce the caregiving burden on families and healthcare institutions.