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OBJECTIVE: Describe the clinical characteristics, treatment strategies, and outcome data of children with papilledema associated with Lyme disease at a large tertiary care pediatric hospital. METHODS: Retrospective cohort study of children 1-18 years old who received care at our institution between 1995 and 2019 with concurrent diagnoses of papilledema and Lyme disease. Data were abstracted from records and prospective family surveys. RESULTS: Among 44 children included (median age 9.7 years), 66% (29/44) had additional cranial neuropathies, and 78% (32/41) had cerebrospinal fluid pleocytosis. All children were treated with antibiotics (39% oral, 55% intravenous, 7% both); 61% (27/44) were also treated with oral acetazolamide. Symptoms fully resolved in 86% (30/35) of children with follow-up data. Proportion recovered did not significantly differ by antibiotic administration route or presence/absence of cerebrospinal fluid pleocytosis. CONCLUSIONS: Papilledema in Lyme disease may occur with or without cerebrospinal fluid pleocytosis. Most children recover without residual deficits following treatment, although exceptions exist.

Decompressive hemicraniectomy (DHC) is a critical procedure used to alleviate elevated intracranial pressure (ICP) in emergent situations. It is typically performed to create space for the swelling brain and to prevent dangerous and potentially fatal increases in ICP. DHC is indicated for pathologies ranging from MCA stroke to traumatic subarachnoid hemorrhage-essentially any cause of refractory brain swelling and elevated ICPs. Scalp incisions for opening and closing the soft tissues during DHC are crucial to achieve optimal outcomes by promoting proper wound healing and minimizing surgical site infections (SSIs). Though the reverse question mark (RQM) scalp incision has gained significant traction within neurosurgical practice, alternatives-including the retroauricular (RA) and Kempe incisions-have been proposed. As choice of technique can impact postoperative outcomes and complications, we sought to compare outcomes associated with different scalp incision techniques used during DHC. We queried three databases according to PRISMA guidelines in order to identify studies comparing outcomes between the RQM versus alternative scalp incision techniques for DHC. Our primary outcome of interest in the present study was postoperative wound infection rates according to scalp incision type. Secondary outcomes included estimated blood loss (EBL) and operative duration. We identified seven studies eligible for inclusion in the formal meta-analysis. The traditional RQM technique shortened operative times by 36.56 min, on average. Additionally, mean EBL was significantly lower when the RQM scalp incision was used. Postoperatively, there was no significant association between DHC incision type and mean intensive care unit (ICU) length of stay (LOS), nor was there a significant difference in predisposition to developing wound complications or infections between the RQM and retroauricular/Kempe incision cohorts. Superficial temporal artery (STA) preservation and reoperation rates were collected but could not be analyzed due to insufficient number of studies reporting these outcomes. Our meta-analysis suggests that there is no significant difference between scalp incision techniques as they relate to surgical site infection and wound complications. At present, it appears that outcomes following DHC can be improved by ensuring that the bone flap is large enough to enable sufficient cerebral expansion and decompression of the temporal lobe, the latter of which is of particular importance. Although previous studies have suggested that there are several advantages to performing alternative scalp incision techniques during DHC, the present study (which is to our knowledge the first to meta-analyze the literature on outcomes in DHC by scalp incision type) does not support these findings. As such, further investigations in the form of prospective trials with high statistical power are merited.

BACKGROUND AND OBJECTIVES: Elevated intracranial pressure (ICP) in myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) has been largely unexplored. The objectives of this study were to determine the frequency of increased ICP in MOGAD and its association with disease course and outcomes and to highlight cases requiring medical and/or surgical management of increased ICP. METHODS: In this retrospective, single-center cohort study, we examined the clinical and paraclinical data from the initial presentation and follow-up data of children diagnosed with MOGAD. In those with opening pressure (OP) measurements, univariate analyses were used to evaluate factors associated with increased ICP, which was defined as OP > 28 cm H2O. We also present a case series of patients with or without OP measurement who required medical and/or surgical management of increased ICP. RESULTS: Of 86 children with MOGAD, 43 (50.0%) had an OP recorded and 7 (8.1%) required ICP management. In those with OP recorded, the median (interquartile range) OP for the different MOGAD phenotypes were: 30.0 (22.8-41.6) (acute disseminated encephalomyelitis, ADEM), 20.5 (16.1-23.6) (optic neuritis), 17.0 (17.0-22.5) (myelitis), and 19.5 (16.5-29.3) (other) cm H20. Overall, 20.9% had increased ICP based on an OP > 28 cm H2O, of whom 77.8% presented with ADEM. In a subgroup analysis of those presenting with ADEM, those with an elevated ICP had longer hospital stay (p = 0.007) and neurologic disability (defined as modified Rankin Scale >1) (p = 0.049). In those with or without OP recorded, 7 (6 with ADEM, one with cerebral cortical encephalitis) required ICP-directed therapies. Findings on brain MRI in these 7 children revealed extensive disease burden with bilateral cerebral involvement and evidence of restricted diffusion. While neuropsychological data in this small subset revealed significant variability, all sustained identifiable deficits after discharge, including attention-deficit hyperactivity disorders and language and learning disorders. DISCUSSION: In pediatric MOGAD, increased OP and ADEM at initial presentation were associated with longer hospital stays and greater long-term morbidity. Although invasive ICP monitoring has not been specifically advocated in the management of MOGAD, it is important to recognize signs and symptoms of increased ICP in these patients and consider ICP monitoring and management strategies based on clinical and radiologic findings, especially in those presenting with ADEM and with OP > 28 cm H2O.

Detecting and monitoring elevated intracranial pressure (ICP) is crucial in managing various neurologic and neuro-ophthalmic conditions, where early detection is essential to prevent complications such as seizures and stroke. Although traditional methods such as lumbar puncture, intraparenchymal and intraventricular cannulation, and external ventricular drainage are effective, they are invasive and carry risks of infection and brain hemorrhage. This has prompted the development of non-invasive techniques. Given that direct, non-invasive access to the brain is limited, a significant portion of research has focused on utilizing the eyes, which uniquely provide direct access to their internal structure and offer a cost-effective tool for non-invasive ICP assessment. This review explores the existing non-invasive ocular techniques for assessing chronically elevated ICP. Additionally, to provide a comprehensive perspective on the current landscape, invasive techniques are also examined. The discussion extends to the limitations inherent to each technique and the prospective pathways for future advancements in the field. [ABSTRACT FROM AUTHOR]

Background and Purpose: Elevated intracranial pressure (ICP) resulting from severe head injury or stroke poses a risk of secondary brain injury that requires neurosurgical intervention. However, currently available noninvasive monitoring techniques for predicting ICP are not sufficiently advanced. We aimed to develop a minimally invasive ICP prediction model using simple CT images to prevent secondary brain injury caused by elevated ICP. Methods: We used the following three methods to determine the presence or absence of elevated ICP using midbrain‐level CT images: (1) a deep learning model created using the Python (PY) programming language; (2) a model based on cistern narrowing and scaling of brainstem deformities and presence of hydrocephalus, analyzed using the statistical tool Prediction One (PO); and (3) identification of ICP by senior residents (SRs). We compared the accuracy of the validation and test data using fivefold cross‐validation and visualized or quantified the areas of interest in the models. Results: The accuracy of the validation data for the PY, PO, and SR methods was 83.68% (83.42%‐85.13%), 85.71% (73.81%‐88.10%), and 66.67% (55.96%‐72.62%), respectively. Significant differences in accuracy were observed between the PY and SR methods. Test data accuracy was 77.27% (70.45%‐77.2%), 84.09% (75.00%‐85.23%), and 61.36% (56.82%‐68.18%), respectively. Conclusions: Overall, the outcomes suggest that these newly developed models may be valuable tools for the rapid and accurate detection of elevated ICP in clinical practice. These models can easily be applied to other sites, as a single CT image at the midbrain level can provide a highly accurate diagnosis. [ABSTRACT FROM AUTHOR]

Ventriculoperitoneal (VP) shunt placement, essential for managing hydrocephalus, often risks catheter malpositioning, especially in patients with small ventricles. We present a novel technique combining neuronavigation with intraoperative cone-beam computed tomography using the BrainLab system and Loop-X mobile imaging unit. This approach enables real-time verification of catheter placement by integrating preoperative MRI data with intraoperative CT imaging. In a 12-year-old boy with therapy-refractory idiopathic intracranial hypertension, neuronavigation was guided by the BrainLab Skull Fix and Cushing canula, ensuring precise catheter insertion into the right frontal horn. Post-placement, Loop-X facilitated immediate verification of the catheter's trajectory and positioning, corroborated by postoperative MRI. This technique demonstrated high precision and minimized radiation exposure, emphasizing its utility in reducing revision rates due to suboptimal catheter placement. [ABSTRACT FROM AUTHOR]

Purpose: We demonstrate the complexities of managing pediatric patients on extracorporeal membrane oxygenation (ECMO) therapy requiring neurosurgery, focusing on systemic anticoagulation, cardiac function, and medically refractory intracranial pressure (ICP). Methods: A 3.5-year-old female with Tetralogy of Fallot developed severe ischemic cerebral edema following post-operative cardiac arrest and required ECMO. This case, along with four additional cases of children requiring neurosurgery while on ECMO, was examined. Results: Emergency neurosurgical intervention in the primary case led to significant improvement, highlighting the delicate balance between managing ECMO-induced anticoagulation and urgent neurosurgical needs. The additional cases had variable outcomes, emphasizing the challenges of caring for these critically ill patients. Conclusion: Successful management of children requiring ECMO support and neurosurgical intervention requires thoughtful multidisciplinary care. This report illustrates some of the nuances in such decision-making, and demonstrates one potential path to a good outcome. [ABSTRACT FROM AUTHOR]

Introduction: The role of decompressive craniectomy (DC) is as a rescue therapy for the treatment of intracranial hypertension. The indications for the DC are variable. Methods: The clinical details, imaging, operative findings and follow-up data of children less than or equal to 18 years of age were reviewed for more information on the children who underwent DC in the last 5 years. Results: During the study period, a total of 128 children underwent DC. The trauma cases were 66, and the non-trauma cases were 62. The common indication for DC was pure acute subdural hematoma 33 (50%), followed by contusion 10 (15%) in the trauma group, and in non-trauma, arterial infarction in 20 (32%) and cerebral venous thrombosis in 17 (27%). Hemicraniectomy was done in 114 (89%), and bifrontal craniectomy was done in 7 (5.4%) cases. The median duration follow-up was 7 months in non-trauma and 6 months in trauma. GCS was less than 8, the motor score was less than 3, and pupillary asymmetry, hypotension and basal cistern effacement were factors related to an unfavourable outcome in the non-trauma group. In regression analysis, only a motor score of less than three was associated with the non-trauma group. Age less than 10 years, GCS less than 8, motor score less than three and preoperative infarction were the predictive factors in univariate analysis, and only GCS less than 8 was the predictive factor for unfavourable factors in regression analysis in the trauma group. Conclusion: The DC is performed as a lifesaving procedure. The unfavourable outcome is slightly higher in non-trauma cases compared to trauma cases. However, the mortality rate is high in trauma cases. [ABSTRACT FROM AUTHOR]

Dural venous sinus stenting is an emerging and exciting area in otolaryngology in collaboration with neurosurgeons and neuroradiologists. The first cases were reported 20 years ago. It is now considered part of the routine treatment of increased intracranial pressure due to transverse sinus stenosis. ENT doctors are the first to see these patients in their clinics, as sinus headaches, pulsating tinnitus, and dizziness are the most common symptoms. Previously, with limited success, high-dose diuretics and intracranial shunts had been the only options for treating these patients. Other methods, such as covering the sigmoid sinuses with graft material, appear to cause a sudden increase in intracranial pressure that can lead to blindness and even death. This overview summarizes the clinical and imaging characteristics of patients who will benefit from endovascular sinus stenting for elevated intracranial pressure. [ABSTRACT FROM AUTHOR]

Topiramate is often considered as a second-line medication for the treatment of pseudotumor cerebri syndrome (PTCS), but limited studies exist that evaluate its efficacy in children. Retrospective study of patients aged <21 years with PTCS who were treated with topiramate alone or in combination with acetazolamide was performed. Data regarding clinical courses and visual outcomes were recorded. A total of 46 patients were identified. Three (6.5%) patients were treated with topiramate alone, 31 (67.4%) transitioned to topiramate from acetazolamide, and 12 (26.1%) took both topiramate and acetazolamide concurrently. The median time to resolution of papilledema on topiramate was 0.57 years (interquartile range 0.32 to 0.84). Among eyes with papilledema graded on the Frisen scale at topiramate initiation, 40 of 57 (70.2%) were grade 1, nine of 57 (15.8%) were grade 2, and eight of 57 (14.0%) were grade 3. Twenty-seven of 46 (58.7%) reported headache improvement after starting topiramate. The mean dose of topiramate was 1.3 ± 0.8 mg/kg/day. The most common side effect was patient report of cognitive slowing (10 of 46 [21.7%]). All patients on topiramate monotherapy who were compliant with treatment and follow-up had resolution of papilledema with no evidence of visual function loss. Topiramate can effectively treat PTCS in children with mild to moderate papilledema or in those unable to tolerate acetazolamide. More research is needed to assess the efficacy of topiramate for higher grade papilledema. [ABSTRACT FROM AUTHOR]

Neurenteric cysts (NECs) are rare congenital, benign lesions of the central nervous system (CNS), predominantly located within the spinal cord. However, they may also occur less frequently within the brainstem, fourth ventricle, or cerebellopontine angle (CPA). Originating from anomalous interactions between embryonic layers, NECs are recognized for their potential to compress adjacent structures. We report a unique case of disseminated NECs exhibiting few to absent symptoms, which represents an unusual presentation of this disease, with only six similar reports in the literature. A 22‐year‐old female presented to our institute with chronic headaches resistant to nonsteroidal anti‐inflammatory drugs (NSAIDs). Initially treated for intracranial hypertension (ICH) secondary to a cyst in the quadrigeminal cistern at the age of 17 via neuroendoscopic surgery and subsequent ventriculoperitoneal shunting, she experienced transient relief. However, follow‐up at 22 years of age revealed multiple cysts in the basal and spinal cisterns, with MRI findings suggestive of neurocysticercosis. Despite treatment with albendazole and corticosteroids, subsequent MRIs showed no change in the size or number of the cysts. Six years later, symptoms had worsened, previously identified cysts had grown, and the detection of new cysts prompted surgical intervention. Histopathological examination confirmed the presence of NECs. This case highlights the diagnostic challenges posed by NECs, especially in regions endemic for neurocysticercosis, where clinical and radiological findings may initially suggest this condition. It underscores the importance of considering NECs in the differential diagnosis of cystic lesions in the CNS, even in the absence of typical symptoms of spinal cord compression. The recurrence and spread of NECs post‐treatment demand a comprehensive management approach, encompassing surgical intervention and close monitoring. [ABSTRACT FROM AUTHOR]

Background: Increased intracranial pressure (ICP) in patients with idiopathic intracranial hypertension (IIH) affects the retinal microvasculature, which can be imaged and quantified by optical coherence tomography angiography (OCTA). We aimed to identify the mediating factor between ICP and OCTA parameters association in IIH patients. Methods: IIH patients with active intracranial hypertension were enrolled. OCTA imaging was performed after ICP measurement. We quantified the branching complexity of the retinal arterioles and venules from the superficial vascular complex of the OCTA image. Eyes of IIH patients were stratified into eyes with papilledema (IIH-P) and eyes without papilledema (IIH-WP). All participants underwent visual acuity (VA) examination. Results: One hundred and thirty-eight eyes from 70 IIH patients and 146 eyes from 73 controls were included. Compared to the control group, IIH patients and IIH-P had reduced arteriole complexity and increased venule complexity (p < 0.05). For IIH patients and IIH-P, increased retinal venule complexity correlated with increased ICP and reduced VA (p < 0.05); while decreased arteriole complexity only correlated with Frisen scores (p = 0.026). Papilledema mediated the effect (p < 0.001) between ICP and arteriole complexity while ICP had a direct effect (p < 0.001) on venule complexity. Conclusion: Retinal venules imaged via OCTA may reflect ICP levels and may underpin the direct effect of increased ICP in IIH patients. [ABSTRACT FROM AUTHOR]

Patients with acute brain injury are vulnerable to secondary deterioration, which may go undetected by traditional monitoring. However, multimodal neuromonitoring of brain tissue oxygen tension (PbtO2) and energy metabolism may be able to detect such episodes. We report a retrospective, observational study of 94 patients with aneurysmal subarachnoid haemorrhage (SAH) or traumatic brain injury (TBI) who underwent multimodal neuromonitoring during admission. We examined the co-occurrence of pathological neuromonitoring values: elevated intracranial pressure (ICP, > 20 mmHg), inadequate cerebral perfusion pressure (CPP, < 60 mmHg), brain hypoxia (PbtO2 < 20 mmHg), and metabolic crisis (lactate/pyruvate ratio > 40 and a glucose level < 0.2 mmol/L in cerebral microdialysate). Mixed effects linear regression demonstrated significant associations between abnormal ICP/CPP, cerebral hypoxia and metabolic crisis. However, brain hypoxia occurred in 40% and 31% of observations in patients with SAH and TBI, respectively, despite normal concurrent values of ICP. Similarly, metabolic crisis was observed in 8% and 16% of measurements for SAH and TBI, respectively, despite a normal ICP. The pattern was identical for CPP. In conclusion, although all neuromonitoring variables are interrelated, brain hypoxia and metabolic crisis are common despite an absence of abnormalities in conventional monitoring. Multimodal neuromonitoring may help identify such episodes and guide individualised treatment. [ABSTRACT FROM AUTHOR]

Acute promyelocytic leukemia (APML) can lead to pseudotumour cerebri (PTC), either due to direct effects of the disease or as a complication of its treatment with the vitamin A derivative all-trans-retinoic acid or arsenic. We report the case of a patient with sight-threatening papilledema secondary to APML-induced PTC who was treated with bilateral optic nerve sheath fenestration. [ABSTRACT FROM AUTHOR]

Background: Preeclampsia is implicated in 14% of maternal deaths worldwide, mostly due to complications such as intracranial hemorrhage and cerebral edema. Cerebral edema increases intracranial pressure, which can be predicted by ultrasonographic measurement of the optic nerve sheath diameter (ONSD). Greater diameters have been reported in women with preeclampsia and eclampsia; however, data are lacking on the possible association with maternal and neonatal adverse outcomes. This study aimed to determine whether there is an association between hypertensive disorders of pregnancy and the ONSD, and between this measurement and maternal and neonatal adverse outcomes. Methods: This was a cohort study involving 183 women in the third trimester of pregnancy or within 24 h following childbirth, distributed as follows: control group (n = 30), gestational hypertension (n = 14), chronic hypertension (n = 12), preeclampsia without severe features (n = 12), preeclampsia with severe features (n = 62), superimposed preeclampsia (n = 23) and eclampsia (n = 30). The participants underwent ocular ultrasonography, and data on maternal and neonatal outcomes were collected from the medical records. To compare the groups, analysis of variance was used for the normally distributed numerical variables and the Kruskal–Wallis test was used for variables with non-normal distribution. Two-tailed p-values ≤ 0.05 were considered statistically significant. Results: Overall comparison between the seven groups showed no statistically significant difference in the mean ONSD (p = 0.056). Nevertheless, diameters were significantly greater in the eclampsia group compared to the control group (p = 0.003). Greater diameters were associated with maternal admission to the intensive care unit (ICU) (p < 0.01) and maternal near miss (p = 0.01). There was no association between ONSD and admission to the neonatal ICU (p = 0.1), neonatal near miss (p = 0.34) or neonatal death (p = 0.26). Conclusions: No association was found between ONSD and the hypertensive disorders of pregnancy in the overall analysis; however, ONSD was greater in women with eclampsia compared to controls. Greater diameters were associated with maternal admission to the ICU and maternal near miss. These findings suggest a potential use for bedside ultrasound as an additional tool for stratifying risk in patients with hypertensive disorders of pregnancy. [ABSTRACT FROM AUTHOR]

Background: One of the most underestimated types of chronic headaches is headaches as a result of cerebro-spinal fluid dysregulation disorders. Idiopathic intracranial hypertension (IIH) presents with headache and visual symptoms and usually is associated with papilledema. We identified patients with IIH in a chronic headache population presenting to a hospital-based headache clinic, and studied its associations with clinical, sonographic and magnetic resonance imaging (MRI) findings. Methods: Of 168 patients, 141 chronic headache patients were identified and completed the study procedures (semi-structured medical interview, fundus examination, MRI brain with magnetic resonance venography (MRV) and trans-orbital sonography (TOS)). Patients with abnormal findings underwent lumbar puncture for opening pressure. Results: The prevalence of IIH was 27%. IIH patients were of higher age, had gnawing/throbbing headache in the vertex in most cases, and had higher body mass index. Fundus examination had a sensitivity of 79% and a specificity of 98% for the detection of IIH cases. Approximately 23% of IIH patients had no papilledema. The most sensitive MR sign was found to be transverse sinus stenosis. TOS showed optic nerve sheath dilation in 35.7% of IIH without papilledema cases. Conclusions: The prevalence of IIH is high in the chronic headache population and should be suspected in the headache clinic setting, particularly because there is significant overlap with migraine symptomatology. MRI/MR venography and TOS can be useful adjunct tests to identify IIH patients. [ABSTRACT FROM AUTHOR]

(1) Background: Neural tube defects are a prevalent cause of congenital malformations, myelomeningocele (MMC) being the most severe form. This study evaluates the clinical outcome and postoperative-associated complications following MMC surgical closures, focusing on the following three techniques: direct suture (DS); VY advancement flap (VYF); and Limberg flap (LF). (2) Methods: A retrospective observational study was conducted from March 2015 to February 2023, and the inclusion criteria were newborns who underwent lumbosacral MMC within 24 h of birth. (3) Results: Out of 20 cases, 45% underwent DS closure; 25% underwent VY-F closure; 15% underwent LF closure, and 15% (n = 3) underwent combined flap closure. A significant statistical correlation was observed between intracranial hypertension (IH), the need for external ventricular drainage (EVD), and DS closure type. In the DS group, 60% of patients required EVD (p = 0.041), and 90% had IH (p = 0.027). CSF fistula was present in 40% of LF cases and 30% of DS cases, while wound dehiscence was observed in 60% of LF cases and 30% of DS cases. (4) Conclusions: Our study demonstrated that DS was linked to higher rates of complications. The VY-F is the safest method for closing MMC defects. [ABSTRACT FROM AUTHOR]