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Background: Hydrocephalus frequently occurs with midline posterior fossa cystic collections. The classification of this heterogeneous group of developmental anomalies, including Dandy-Walker malformation, persisting Blake's pouch, retrocerebellar arachnoid cysts, and mega cisterna magna, is subject of debate. The absence of diagnostic criteria is confusing regarding the ideal management of PFCC-related hydrocephalus., Objective: To decipher the surgical strategy for the treatment of children with PFCC-related hydrocephalus through a retrospective analysis of the surgical outcome driven by their clinical and radiological presentation., Methods: This study enrolled patients operated of symptomatic PFCC-related hydrocephalus. Clinical and MRI features were examined, as well as the surgical outcome. Unbiased subgroup classification of the patients was performed with multiple component analysis as a function of imaging characteristics and hierarchical clustering on principal component. Outcome was assessed with binomial logistic regression and Kaplan-Meier analysis., Results: Fifty-four patients were included between 2007 and 2021. Multiple component analysis suggested that cerebellar and vermian hypoplasia, vermian rotation, basal-tentorial angle, and fastigial angle were strongly correlated. Hierarchical clustering and the distribution of the patients in the bidimensional plot showed the clear segregation of 3 major clusters, which correlated with the radiological diagnosis ( P < .01). Binomial logistic regression and survival analysis showed that endoscopic third ventriculostomy was an effective treatment for patients with persisting Blake's pouch, while failing to control hydrocephalus in most of patients with Dandy-Walker malformation., Conclusion: Preoperative MRI in patients with PFCC-related hydrocephalus is essential to better define the diagnosis. The choice of treatment strategy notably relies on correct radiological diagnosis., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Background: Focal cortical dysplasia (FCD) causes drug-resistant epilepsy in children that can be cured surgically, but the lesions are often unseen by imaging., Objective: To assess the efficiency of arterial spin labeling (ASL), voxel-based-morphometry (VBM), fMRI electroencephalography (EEG), resting-state regional homogeneity (ReHo), 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET), and their combination in detecting pediatric FCD., Methods: We prospectively included 10 children for whom FCD was localized by surgical resection. They underwent 3T MR acquisition with concurrent EEG, including ASL perfusion, resting-state BOLD fMRI (allowing the processing of EEG-fMRI and ReHo), 3D T1-weighted images processed using VBM, and FDG PET-CT coregistered with MRI. Detection was assessed visually and by comparison with healthy controls (for ASL and VBM)., Results: Eight children had normal MRI, and 2 had asymmetric sulci. Using MR techniques, FCD was accurately detected by ASL for 6/10, VBM for 5/10, EEG-fMRI for 5/8 (excluding 2 with uninterpretable results), and ReHo for 4/10 patients. The combination of ASL, VBM, and ReHo allowed correct FCD detection for 9/10 patients. FDG PET alone showed higher accuracy than the other techniques (7/9), and its combination with VBM allowed correct FCD detection for 8/9 patients. The detection efficiency was better for patients with asymmetric sulci (2/2 for all techniques), but advanced MR techniques and PET were useful for MR-negative patients (7/8)., Conclusion: A combination of multiple imaging techniques, including PET, ASL, and VBM analysis of T1-weighted images, is effective in detecting subtle FCD in children., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)

Background: Multiple burr hole (MBH) surgery is a simple, safe, and effective indirect technique of revascularization in moyamoya angiopathy (MM). However, it is not yet recognized as a first-line treatment., Objective: To assess the long-term outcome and perioperative complications in a large single-center cohort of children with MM who underwent burr hole surgery., Methods: This study is a retrospective analysis of children who underwent surgery for MM in a national reference center for pediatric stroke between 1999 and 2015. Sixty-four children (108 hemispheres, median age 7 years) were consecutively treated. The indication for revascularization was previous stroke or transient ischemic attack (TIA) or rapidly progressive disease on brain magnetic resonance imaging (MRI) and digital subtraction angiography. Children were followed with clinical examinations, telephone interviews, and MRI with any clinical recurrence of stroke or TIA used as the primary endpoint. Surgical mortality and morbidity were documented., Results: Sixty-four patients were operated (bilateral MBH n = 39, unilateral procedure n = 25). At a mean follow-up of 4.2 years and 270.6 patient years, 89.1% of patients had not suffered any recurrent stroke or TIA. A second surgery was required in 5 cases after unilateral revascularization, and in 3 cases after bilateral MBH. Mortality associated with the procedure was 0. Postoperative Matsushima angiographic grading was the only predictive factor of ischemic recurrence ( P = .036)., Conclusion: In pediatric MM, MBH compares favorably to other indirect or direct revascularization techniques in children in the prevention of stroke or TIA., (Copyright © 2017 by the Congress of Neurological Surgeons)

Background: Suprasellar arachnoid cysts (SAC) represent between 9% and 21% of pediatric arachnoid cysts. Recent improvements in magnetic resonance imaging, as well as increasing prenatal diagnosis, have allowed more precise knowledge and follow-up., Objective: To describe a novel classification of SAC., Methods: We present 35 cases of SAC treated between 1996 and 2014. Patient records and imaging studies were reviewed retrospectively to assess symptomatology, radiological findings, treatment, and long-term follow-up., Results: Fourteen SAC were diagnosed prenatally (39%). We observed 15 (43%) cases presenting hydrocephalus (SAC-1) removing Liliequist membrane downward. Lower forms (SAC-2) with free third ventricle were observed in 11 (31%) cases. Asymmetrical forms (SAC-3) with Sylvian or temporal extension were seen in the 9 (26%) remaining patients. Twenty-three (66%) patients were treated by ventriculocisternostomy, 3 (8.5%) by shunt surgery, and 3 (8.5%) by craniotomy. Six (17%) patients had no surgery, including 5 cases (14%) that had prenatal diagnosis. Outcomes were initially favorable in 26 cases (87%). Eight (22%) patients had endocrine abnormalities at the end of the follow-up, 3 (8.5%) had developmental delay, and 6 (17%) had minor neuropsychological disturbances., Conclusion: SAC are heterogeneous entities. SAC-1 may come from an expansion of the diencephalic leaf of the Liliequist membrane. SAC-2 show a dilatation of the interpeduncular cistern and correspond to a defect of the mesencephalic leaf of the Liliequist membrane. SAC-3 correspond to the asymmetrical forms expanding to other subarachnoid spaces. Surgical treatment is not always necessary. The recognition of the different subtypes will allow choosing the best treatment option.

Background: Symptomatic posterior fossa hematoma in the term newborn is rare., Objective: To report on the management and outcome of posterior fossa subdural hematoma (PFSDH) in neonates., Methods: A retrospective analysis of the department database and clinical notes of neonates admitted since 1985 with a PFSDH was performed together with a literature review of similar case series., Results: Sixteen patients were included. The median gestational age was 40 weeks with a high proportion of primiparous mothers (n = 9) and forceps delivery (n = 9). Nine neonates had symptoms of brainstem dysfunction within the first 24 hours of life, whereas the other 7 had a delayed presentation (median 4 days) with signs of raised intracranial pressure due to hydrocephalus. Each patient had a cranial ultrasound followed by computed tomography scan that showed the PFSDH. Eleven neonates required surgical evacuation of the PFSDH, whereas hydrocephalus was managed by transient external ventricular drainage in 2 further patients. Eventually, 2 neonates required a permanent ventriculoperitoneal shunt. Five neonates had no operative intervention. With a mean follow-up of 7.8 years, 2 patients had mild developmental delay and 1 had severe developmental delay. The 13 other patients had a normal development., Conclusion: In neonates with a PFSDH, surgery can be safely performed in those who have clinical and radiological signs of brainstem compression or hydrocephalus. A small number of neonates require a ventriculoperitoneal shunt in the long term. Initial aggressive resuscitation should be performed even in cases of initial severe brainstem dysfunction because of the good long-term neurological outcome.

Background: In cryptogenic epilepsy or when multifocal seizure onset is suspected, intracranial monitoring of the EEG is required., Objective: To report on the adverse events related to electroencephalogram (EEG) intracranial recording in one of the largest pediatric series published and to discuss the avoidance of adverse events in our experience and with respect to a review of the literature., Methods: A retrospective analysis of our department database and hospital charts of 95 children operated on between 1994 and 2009 was performed., Results: Invasive recording was uneventful in 51.1% of cases. Observed frequency of infection was 14.9%, cerebrospinal fluid leak was 10.6%, brain swelling was 6.4%, and hemorrhage was 17%. Brain swelling was more frequent in older patients, whereas the length of recording, number of electrode contacts used, and presence of depth electrodes were not relevant. Cerebrospinal fluid leakage was completely prevented by the routine introduction of dural graft substitutes in 2003., Conclusion: Invasive recordings carry a noticeable rate of adverse events but provide invaluable information in delineating the epileptogenic zone. The low incidence of such events among younger children suggests that invasive recordings can be successfully performed with low morbidity in this age group.

Background: Cortical stimulation is under investigation in clinical trials of drug-resistant epilepsy. Results are heterogeneous; therefore, more evidence from animal studies is required., Objective: To investigate the therapeutic effects of parameters of direct stimulation of the cortical focus in a Macaca fascicularis presenting focal motor epilepsy., Methods: We developed a model of motor seizures after intracortical injection of penicillin G in the primary motor cortex of a Macaca fascicularis. We performed electric epidural cortical stimulation at low, medium, and high frequency using continuous or short-term stimulation. Short-term stimulation was triggered on seizure onset, either visually or automatically with a seizure detection algorithm connected to a programmable stimulator., Results: Automated detection could detect 100% of the seizures, but ensuing cortical electric stimulation failed to abort seizures., Conclusion: This study demonstrates the inefficacy of the stimulation of the cortical focus to prevent seizures induced by local injection of penicillin G. Because this model may be too severe to allow comparison to human epilepsies, further work is required in other monkey models of focal epilepsy.

Background: Subdural collection is a possible adverse event that can occur after hemispherotomy., Objective: : We describe our surgical technique to avoid subdural collection after peri-insular hemispherotomy in children., Methods: Since 2008, we have closed the corticotomy with interrupted pial sutures and fibrinogen glue in 16 patients., Results: This operative technique stopped the occurrence of postoperative subdural collection and was not associated with an increased incidence of postoperative hydrocephalus., Conclusion: We recommend closing ventricular access after functional hemispherotomy, notably in patients who have had a stroke.