Child's Nervous System最新文献

Purpose: Treatment of subjects with refractory idiopathic intracranial hypertension (IIH) or shunted hydrocephalus with chronic shunt complications is challenging. What is the role for cranial vault expansion, particularly utilizing posterior vault distraction osteogenesis (PVDO), in these cases? This study assesses medium-term efficacy of cranial vault expansion in this unique patient population.

Methods: A retrospective review was conducted of patients who underwent cranial vault expansion from 2008 to 2023 at the Children's Hospital of Philadelphia. Subjects who did not have a diagnosis of primary craniosynostosis were included in the study. Demographic information, medical history, and perioperative details were collected from medical records. Primary outcomes were the rate of CSF diversion procedures and resolution of presenting signs and symptoms. Secondary outcomes were perioperative and 90-day complications and reoperation requirement.

Results: Among 13 included subjects, nine (69.2%) patients had a primary diagnosis of shunted hydrocephalus and 4 (30.8%) patients had IIH. Twelve (92.3%) subjects underwent posterior vault distraction osteogenesis (PVDO) and one (7.7%) underwent posterior vault remodeling (PVR). All 4 patients with IIH demonstrated symptomatic improvement following PVDO, including resolution of headaches, vomiting, and/or papilledema. Among 9 patients with shunted hydrocephalus, CSF diversion requirement decreased from 2.7 ± 1.6 procedures per year preoperatively to 1.2 ± 1.8 per year following cranial vault expansion (p = 0.030). The mean postoperative follow-up was 4.1 ± 2.1 years and four (30.8%) patients experienced complications within 90 days of surgery, including infection (n = 2), CSF leak (n = 1), and elevated ICP requiring lumbar puncture (n = 1). Four (30.8%) patients underwent repeat cranial vault expansion for recurrence of ICP-related symptoms. At most recent follow-up, 7 of 9 patients with shunted hydrocephalus demonstrated symptomatic improvement.

Conclusion: Cranial vault expansion reduced intracranial hypertension-related symptomology as well as the rate of CSF diversion-related procedures in patients with refractory IIH and shunted hydrocephalus without craniosynostosis, and should be considered in those who have significant shunt morbidity.

Introduction: Traumatic extradural haematoma (EDH) is one of the neurosurgical emergencies in the paediatric population. One of the rare complications of extradural haematoma is adjacent parenchymal infarct from the compressive effect of haematoma leading to further morbidity. We aimed to evaluate the incidence and outcomes of this rare complication in paediatric trauma patients.

Methods: We undertook a retrospective review of all operative extradural haematoma cases in single centre paediatric neurosurgical unit between the years 2008 and 2024. Cases with postoperative imaging were analysed for demographics, age, sex, dimensions of haematoma and underlying infarct, pre-operative GCS and pupillary abnormality and time interval from scan to surgery. Infarct was defined as well-defined parenchymal hypodensity corresponding to vascular territory and not attributable to contusions on postoperative CT head performed at 24-72 h. All patients suspected of stroke had further MRI brain for evaluation. Statistical analysis was then performed comparing groups with and without infarct.

Results: Overall, 115 cases of extradural haematoma were identified during the study period. Eighty cases had adequate postoperative imaging to allow assessment for infarcts; 7.5% demonstrated evidence of infarct underlying extradural haematoma. Mean age in the infarct group was 1.8 years (M:F ratio 2:1) with mean EDH dimensions of 77 mm × 31.4 mm × 79.7 mm). Mean age in the non-infarct group was 8.6 years (M:F ratio 2:1) with mean EDH dimensions of (57.5 mm × 19.6 mm × 62.6 mm). Children with infarcts were significantly younger with larger haematoma dimensions and had a higher proportion of pupillary abnormalities. There was no significant difference in pre-operative GCS and scan-to-surgery time-lapse between the groups with and without infarct.

Conclusion: Compressive effect of EDH can lead to underlying parenchymal infarct. Children with extradural haematoma-related infarct were younger with larger haematoma size and a higher proportion of pupillary abnormalities.

Purpose: Pre-operative fourth ventricle roof angle (FVRA) has recently been highlighted as a novel radiographic predictor of clinical severity in Chiari type-I malformation (CM-I) as reported by Seaman et al. (J Neurosurg Pediatr 25:1-8, 2021). This has led to suggestions that FVRA could be included in algorithms to determine indications for surgery. We aimed to test the accuracy of FVRA as a predictor of clinical severity and its effect on post-operative outcome in a large retrospective cohort of paediatric patients who underwent decompression for CM-I.

Methodology: Patients undergoing craniocervical decompression for CM-I at the Queensland Children's Hospital and Mater Hospital, Brisbane, between 2006 and 2018 were included. Data was collected from 66 patients aged 0-18 years. Post-operative outcome was assessed by calculating Chicago Chiari Outcomes Score (CCOS) at follow-up. The FVRA was taken from a mid-sagittal T1-weighted MRI as previously described (Seaman et al. in J Neurosurg Pediatr 25:1-8, 2021). The angle is subtended between superior and inferior medullary velum.

Results: Whilst results from Seaman et al. demonstrated a strong correlation between a FVRA > 65° and symptomatic CM-I, this did not translate to our dataset (p = 0.61). Additionally, pre-operative FVRA is not useful as a predictive tool for post-surgical outcome as assessed by CCOS (p = 0.50), and post-operative reduction in FVRA did not correlate with improved outcomes (p = 0.81). We did note significantly worse outcomes in patients presenting with pre-operative brainstem dysfunction (p = 0.03).

Conclusion: Paediatric CM-I is a challenging cohort to manage, often due to young age and a lack of language skills. There is a heavy reliance on radiological findings. Whilst FVRA has previously been reported to be of value as a determinant of clinical severity in adult and paediatric patients, we find that this measurement is of dubious value in our retrospective cohort.

Purpose: An inflammatory cascade associated with the systemic neutrophil response can be triggered after traumatic brain injury (TBI), causing neuronal dysfunction, which is considered to be related to the prognosis of the victims. The scope of this research is to identify the performance of the neutrophil-lymphocyte ratio (NLR) as a predictor of prognosis considering TBI severity and death as outcomes in a group of pediatric patients.

Methods: We retrospectively evaluated NLR through a consecutive review of the medical records (cross-sectional study) of children and adolescents aged < 17 years victims of TBI. To determine the highest NLR value identified as a predictor, different cutoff points were tested for each outcome. The cutoff points were defined based on the area under curve (AUC) of the receiver operating characteristic (ROC).

Results: Among the 82 children with TBI included in the sample, the performance of AUC-ROC was 0.72 when evaluating NLR as a predictor of TBI severity, with NLR cutoff point of 3, and 0.76 when considering mortality as the outcome, with an increase in the cutoff point to 11.

Conclusion: NLR can be considered a biomarker of brain injury in children and adolescent victims of TBI. Patients with NLR ≥ 3 had a fivefold higher probability of severe TBI and patients with NLR ≥ 11 experienced a ninefold higher risk of death.

Purpose: Unresectable pediatric low-grade gliomas (LGG) usually need adjuvant therapy, and carboplatin hypersensitivity reaction (HR) commonly leads to premature treatment cessation of a standard chemotherapy regimen. In the molecular era, advances in understanding tumor genetic characteristics allowed the development of targeted therapies for this group of tumors; however, cost-effectiveness assessment of treatments, especially in low-income countries, is crucial. The aim is to describe the results of carboplatin desensitization protocol in a single center in a middle-income country.

Method: Prospective analysis of children with LGG submitted to carboplatin desensitization from December 2017 to June 2020 with follow-up until April 2024.

Results: Nine patients were included. The mean age was 11 years. Five patients were male. Seven had optic pathway and two cervicomedullary location. Six had histologic diagnosis and four molecular analyses. The incidence of carboplatin reactions during the study period was 39.1%. Six patients underwent skin prick test, three with positive results. The first HR occurred, on average, around the 9th cycle of treatment. All patients had cutaneous symptoms, and five out of nine had anaphylaxis as the first reaction. 77.7% of the patients completed the protocol, and the clinical benefit rate (stable disease and partial response) was 88.8%. Six patients further required other lines of therapy. Monthly, the total cost for carboplatin was $409.09, and for target therapies (dabrafenib plus trametinib), $4929.28 to $5548.57.

Conclusion: Our study presented an interesting and cost-effective option where desensitization allowed children with HR to be treated with first-line therapy, avoiding the discontinuation of an effective treatment.

Purpose: The most common cause of cerebral vasospasm is subarachnoid hemorrhage, less frequently it occurs after trauma, infection, and tumor resection. Vasospasm in children is rare and has not been systematically investigated in posterior fossa surgery.

Methods: The authors undertook a single-center retrospective study of all the pediatric patients who underwent surgery on the posterior fossa and presented with postoperative symptomatic vasospasm in the period from January 2018 to February 2024. Subsequently, a systematic literature review in accordance with the PRISMA guidelines was performed in the PubMed and Scopus databases to identify the published papers on symptomatic vasospasm after posterior fossa surgery in children.

Results: Of the 178 patients who underwent surgery on the posterior fossa, only one patient was diagnosed with symptomatic diffuse vasospasm on postoperative day 21. The systematic literature review provided further 9 children. The underlying pathology comprised 8 intra-axial lesions with 4 medulloblastomas, 1 schwannoma in the medulla oblongata, 1 pilocytic astrocytoma, 1 primitive neuroectodermal tumor, and 1 arteriovenous malformation. The extra-axial lesions were 1 hypoglossal schwannoma and 1 oculomotor nerve schwannoma.

Conclusion: Iatrogenic symptomatic vasospasm after posterior fossa surgery in children is a rare complication with an outcome ranging from complete recovery to the death of the patient. It is important for all staff involved in the care of patients undergoing surgery on the posterior fossa to be aware of this rare postoperative complication. The small number of patients affected does not allow a substantiated conclusion to be drawn about predictive risk factors.

Purpose: Transitional care (TC) is increasingly crucial, particularly in neurosurgery, where optimal follow-through of patients' care is paramount. Challenges of transition are exacerbated by the complexity of neurosurgical conditions, with pediatric and adult neurosurgery often managed separately by different attending neurosurgeons or in different institutions. While numerous models for transitioning have been proposed, several barriers persist, impeding successful transfer from pediatric to adult settings. Our review focuses on important roles neurosurgeons can play in facilitating successful transition, exploring some existing TC models, with emphasis on the benefits of maintaining a single provider.

Methods: Clinic visits data between 2019 to 2023 of patients between the ages of 16 to 26 was compiled retrospectively. Successful transition was defined as continued follow-up moving from pediatric to adulthood with lost to follow-up being that of unsuccessful transition. Age, diagnosis and whether patients were successfully transitioned or lost to follow-up were documented.

Results: 1829 neurosurgical patients between the ages of 16 to 26 were identified over 5 years. A snapshot review identified 78 adolescent patients deemed to require follow-up into adulthood. 13 patients had epilepsy, 32 central nervous system (CNS) tumors, 17 congenital conditions, 14 neurovascular, and 2 patients had idiopathic intracranial hypertension. All 78 were noted to have successfully transited into their adulthood (age 21 and beyond). Having the same providers; having pediatric and adult neurosurgery within the same institution, was the single most important factor in facilitating successful transition.

Conclusion: Neurosurgeons in institutions, particularly those with experience and competencies in both pediatric and adult care, can serve as crucial anchors during the transitional period. At our institution, the implementation of this continuity of care model has demonstrated remarkable success. Institutions with both pediatric and adult services would be uniquely positioned to develop and implement effective transitional care.

Background: Basal ganglia germ cell tumor (BGGCT) is a rare central nervous system (CNS) tumor. Diffuse hemispheric gliomas, H3 G34-mutant (DHGs) is an invasive glioma involving the cerebral hemispheres. The diagnosis of DHGs depends on the integration of histopathology and molecular pathology.

Case report: We reported a patient with an initial diagnosis of BGGCT that was sensitive to subsequent chemoradiotherapy. Unfortunately, a second high-grade glioma was found on magnetic resonance imaging (MRI) six years later. Subsequently, the tumor was completely removed after surgery and the following histopathology plus next generation sequencing (NGS) testing confirmed the diagnosis of DHGs. Interestingly, we found a germline likely pathogenic variant in FANCA. After surgery, the patient received Stupp regimen. The patient had a relapse 13 months after the Stupp regimen and was doing well after surgery.

Conclusions: This is the first report of a patient with heterochronous double primary tumor of BGGCT followed by DHGs.

Background: Non-hypothalamic glioneural hamartomas are rare entities known to cause medically refractory epilepsy. Olfactory bulb hamartomas, in particular, are exceptionally rare.

Methods: We describe a case of an olfactory bulb hamartoma that was surgically resected at our institution. We also performed a literature review of all glioneural hamartomas and discuss the clinical presentation, diagnosis, and management of these lesions.

Results: Herein, we present the unusual case of a typically developing 17-year-old boy with a near life-long history of drug-resistant epilepsy, found to have a 0.8 × 1.0 cm right olfactory bulb hamartoma. Endoscopic endonasal trans-cribriform resection of the lesion led to seizure freedom in the 6-month follow-up period (Engel class 1 outcome). Comprehensive literature review revealed only one other sporadic case, which was also successfully treated with total surgical resection.

Conclusions: Our case of an olfactory bulb hamartoma adds to the limited literature currently available, illustrating key clinical characteristics of these exceedingly rare lesions and outlining an effective, minimally invasive, and low-morbidity treatment strategy.