Stephanie Greene

BACKGROUND: Sacrifice of the internal cerebral vein (ICV) during neurosurgical procedures is exceedingly rare and poorly characterized in the literature. We report the case of a 14-year-old male who sustained an intraoperative right ICV sacrifice during resection of a pineal region epidermoid cyst. OBSERVATIONS: Postoperatively, the patient developed bilateral thalamic infarcts and transient neurological deficits, including left hemiparesis, mutism, and dysphagia. Despite these initial postoperative findings, he experienced significant recovery with supportive care and rehabilitation. Three months after the procedure, he had returned to school with no deficits other than trace triceps weakness, which was completely resolved by 8 months after the procedure. LESSONS: This case provides rare insight into the natural history of ICV infarction and suggests that meaningful recovery may be possible under select conditions. https://thejns.org/doi/10.3171/CASE2686

BACKGROUND: Drug-resistant epilepsy (DRE) following treatment for atypical teratoid rhabdoid tumor (ATRT) is rare. The authors report a case in which hemispherotomy was performed to palliate new-onset DRE after ATRT therapy. OBSERVATIONS: A 6-year-old boy with a large left thalamic ATRT underwent subtotal resection, chemotherapy, and radiation therapy. His neurological baseline remained stable, with right hemiparesis, right hemifield neglect, and apraxia of speech. Two years after tumor recurrence and treatment, he developed frequent tonic seizures of left hemispheric origin. Despite trials of multiple antiseizure medications, the seizures persisted. Evaluation revealed left hemispheric seizure onset and hypometabolism. Given his existing deficits and unilateral disease, the potential benefits of hemispherotomy were determined to outweigh the risks. The patient underwent a left peri-insular hemispherotomy and has remained seizure free off medication without worsened neurological function. LESSONS: In carefully selected patients with aggressive tumor, unilateral seizure onset and preexisting deficits, hemispherotomy can be a well-tolerated and effective treatment for DRE, even in nontraditional contexts such as ATRT. This case highlights the importance of weighing surgical risks against functional baseline when considering epilepsy surgery in pediatric tumor cases. https://thejns.org/doi/10.3171/CASE24728

OBJECTIVE: Arteriovenous malformations (AVMs) are the most common cause of spontaneous intracranial hemorrhage (ICH) in children, often leading to devastating complications. The current literature from the adult AVM population suggests that both younger age and associated aneurysms carry an increased risk of hemorrhagic presentation. However, detailed analysis of pediatric AVM-associated aneurysms and their link to ICH is relatively unknown, with the literature largely consisting of case reports. This study aimed to determine whether AVM-associated aneurysms predispose pediatric patients to ICH. METHODS: A retrospective cohort study of 238 pediatric patients with AVMs who presented to the Children’s Hospital of Pittsburgh from 1988 to 2023 was performed. Hospital records, patient charts, and radiographic imaging studies were reviewed for patient demographic characteristics, presentation status, and AVM architecture. RESULTS: Of the 238 total patients, 44 (18.5%) children with AVM had associated aneurysms. There were 19 (38.8%) feeding artery aneurysms, 8 (16.3%) intranidal aneurysms, 21 (42.9%) postnidal aneurysms, and 1 (2.0%) unrelated aneurysm of 49 aneurysms. Five patients had venous varices. One hundred forty (58.8%) children presented with hemorrhage. Twenty-one of 44 (47.7%) patients with an AVM-associated aneurysm presented with hemorrhage, whereas 119 of 194 (61.3%) with a solitary AVM presented with hemorrhage (p = 0.10). On multivariate analysis, postnidal aneurysm (OR 0.36, p = 0.037) and an increased number of draining veins (OR 0.66, p = 0.049) were significantly associated with a decreased likelihood of hemorrhagic presentation. Deep venous drainage was associated with an increase in hemorrhagic presentation (OR 2.25, p = 0.0045) on multivariate analysis. CONCLUSIONS: Approximately one-fifth of children with AVMs in this study had accompanying aneurysms, and in this patient population, those with postnidal aneurysms and increased number of draining veins had a decreased incidence of hemorrhage on presentation. Feeding artery and intranidal aneurysms were not associated with an elevated risk of hemorrhagic presentation.

OBJECTIVE: Cerebral cavernous malformation (CCM) is a subtype of the vascular malformations found within the cerebral cortex. Although rare and usually discovered incidentally, these vascular abnormalities can predispose patients to spontaneous cerebral hemorrhage and subsequently lead to a myriad of neurological symptoms at presentation such as seizures and other focal neurological deficits. Although the symptoms and presentations of CCM have been adequately described in the adult population, disease characteristics and outcomes have not been extensively described in the pediatric population. Furthermore, the etiology of CCM—e.g., familial versus sporadic disease, as well as the risk factors for hemorrhage and neurological deficits and predictors of clinical and surgical outcomes—has not been adequately explored in the pediatric population. The current study attempts to classify and characterize differences in the clinical presentation, characteristics, and outcomes of CCMs between familial and sporadic cases within the pediatric population. METHODS: A retrospective review identified 131 pediatric patients with radiographically confirmed diagnosis of CCM. All pertinent demographic and clinical variables were collected. CCM lesions were characterized using T2-weighted and susceptibility-weighted angiography (SWAN) MRI. Statistical analysis was conducted using the t-test for continuous variables, whereas categorical variables were analyzed with the Fisher exact test or chi-square test. Multivariate analysis was performed using a Cox proportional hazards model with R version 4.2.0. RESULTS: This retrospective study identified 131 pediatric CCM patients with a mean age of 8.4 years, and 54% (n = 71) were male. Twenty-seven percent (n = 35) were identified as cases with familial CCM, with the remainder classified as sporadic. The most common symptoms at presentation included generalized symptoms (headaches, nausea, and vomiting) or seizures, with a large proportion of patients also presenting as asymptomatic. No significant differences were observed in severity of symptoms between patients harboring different forms of the disease. Patients with familial CCM were noted to have a larger lesion size on average (5.26 cm³ vs 1.6 cm³, p = 0.047). These patients also had a shorter progression-free follow-up interval, with 50% of patients showing progression by 888 days, compared with only 15% of sporadic CCM patients during the same period (p = 0.0019). Familial etiology of the disease and larger average lesion volume were independent, significant predictors of disease progression (p = 0.001, HR 3.29, 95% CI 1.65–6.54) and future hemorrhage (p = 0.023, HR 1.1, 95% CI 1.01–1.10), respectively. CONCLUSIONS: Familial and sporadic CCMs tend to present with similar characteristics within the pediatric population. Patients with the familial form of the disease have an increased risk of progressive disease in terms of further hemorrhagic events.

Background: Minimally invasive approaches to the anterior cranial fossa have evolved over the past few decades. The management of frontal epidural abscesses (EDAs) secondary to diffuse sinusitis in the pediatric population using minimally invasive techniques is scarcely reported in the literature. Herein, we report the utilization of a minimally invasive eyebrow approach for multidisciplinary concurrent evacuation of frontal EDA secondary to diffuse sinusitis and trephination of the frontal sinus in three pediatric patients. Case Reports: Three pediatric patients presented to the emergency room with severe headaches, visual changes, somnolence, and significant facial and periorbital swelling. Imaging revealed diffuse sinusitis with focal frontal epidural extension. In all cases, progressive clinical deterioration along with the radiographic findings mandated urgent surgical intervention. The eyebrow approach allowed for concomitant evacuation of the frontal EDA and trephination of the frontal sinus followed by functional endoscopic sinus surgery in the same setting. All patients tolerated the procedure well with complete resolution of their symptoms at the completion of antibiotic therapy and complete resolution of the EDA. Conclusion: The eyebrow approach is a minimally invasive technique that should be considered as part of the armamentarium in the management of select EDA in the pediatric population. It allows for multidisciplinary collaboration between neurosurgeons and otolaryngologists for concomitant evacuation of the EDA and trephination of the frontal sinus. This approach is a feasible, safe, and effective minimally invasive technique that can be employed for the management of EDA secondary to diffuse sinusitis in the pediatric population.