Management of Pediatric Cavum Cysts: A Scoping Review and a Single Institution Case Series.

INTRODUCTION: Pediatric cavum cysts are a rare yet complicated pathology to manage. The literature is scarce, primarily consisting of case series, and lacking a consensus regarding clear management. In this scoping review, we aimed to compile existing information in the literature regarding the management of pediatric cavum cysts across the last 10 years. We also present our management of 19 patients, the largest case series to date, highlighting knowledge gaps surrounding the management of this salient pathology. METHODS: A literature search using PubMed and SCOPUS was conducted using the following search terms: (pediatric) AND (Cavum septum pellucidum) OR (cavum vergae) OR (cavum velum interpositum) AND (management). Eligibility criteria included peer-reviewed publication published in the last 10 years, pediatric population, cavum cyst, and English language. A retrospective search was conducted for all pediatric cavum cysts between 2013 and 2023 at our institution. Clinical and radiographic characteristics as well as intervention and outcome data were collected for both the scoping review and our cases. RESULTS: 330 total articles were populated using our search. 12 articles met our inclusion criteria. 41.7% (n = 5) of the articles were case series, 33.3% (n = 4) were case reports, 8.3% (n = 1) was a technical article, 8.3% (n = 1) was a systematic review, and 8.3% (n = 1) was a case questionnaire. Resolution of symptoms was noted in all articles of our scoping review, regardless of treatment modality. The average age in our case series was 9.84 years old and average age at diagnosis was 5.53 years old. 6 patients (31.6%) were female and 13 patients (68.4%) were male. 2 out of the 19 patients (10.5%) were surgically treated. CONCLUSION: There is no clear consensus on the management of cavum cysts. A prospective, multicenter study is needed to create standardized pediatric cyst management guidelines. The current thought is that surgical intervention should be saved for those patients with obstructive hydrocephalus and signs of intracranial hypertension.

A role for P-selectin and complement in the pathological sequelae of germinal matrix hemorrhage.

BACKGROUND: Germinal matrix hemorrhage is a devastating disease of pre-term infancy commonly resulting in post-hemorrhagic hydrocephalus, periventricular leukomalacia, and subsequent neurocognitive deficits. We demonstrate vascular expression of the adhesion molecule P-selectin after GMH and investigate a strategy to specifically target complement inhibition to sites of P-selectin expression to mitigate the pathological sequelae of GMH. METHODS: We prepared two fusion proteins consisting of different anti-P-selectin single chain antibodies (scFv's) linked to the complement inhibitor Crry. One scFv targeting vehicle (2.12scFv) blocked the binding of P-selectin to its PSGL-1 ligand expressed on leukocytes, whereas the other targeting vehicle (2.3scFv) bound P-selectin without blocking ligand binding. Post-natal C57BL/6 J mice on day 4 (P4) were subjected to collagenase induced-intraventricular hemorrhage and treated with 2.3Psel-Crry, 2.12Psel-Crry, or vehicle. RESULTS: Compared to vehicle treatment, 2.3Psel-Crry treatment after induction of GMH resulted in reduced lesion size and mortality, reduced hydrocephalus development, and improved neurological deficit measurements in adolescence. In contrast, 2.12Psel-Crry treatment resulted in worse outcomes compared to vehicle. Improved outcomes with 2.3Psel-Crry were accompanied by decreased P-selectin expression, and decreased complement activation and microgliosis. Microglia from 2.3Psel-Crry treated mice displayed a ramified morphology, similar to naïve mice, whereas microglia in vehicle treated animals displayed a more ameboid morphology that is associated with a more activated status. Consistent with these morphological characteristics, there was increased microglial internalization of complement deposits in vehicle compared to 2.3Psel-Crry treated animals, reminiscent of aberrant C3-dependent microglial phagocytosis that occurs in other (adult) types of brain injury. In addition, following systemic injection, 2.3Psel-Crry specifically targeted to the post-GMH brain. Likely accounting for the unexpected finding that 2.12Psel-Crry worsens outcome following GMH was the finding that this construct interfered with coagulation in this hemorrhagic condition, and specifically with heterotypic platelet-leukocyte aggregation, which express P-selectin and PSGL-1, respectively. CONCLUSIONS: GMH induces expression of P-selectin, the targeting of which with a complement inhibitor protects against pathogenic sequelae of GMH. A dual functioning construct with both P-selectin and complement blocking activity interferes with coagulation and worsens outcomes following GMH, but has potential for treatment of conditions that incorporate pathological thrombotic events, such as ischemic stroke.

Non-sedated fast spine magnetic resonance imaging in pediatric patients.

BACKGROUND: Traditional spine magnetic resonance imaging (MRI) protocols require sedation in young children and uncooperative patients. There is an increased interest in non-sedated pediatric MRI protocols to reduce risks associated with anesthetic agents and improve MRI access. OBJECTIVE: To evaluate the image quality of pediatric non-sedated fast spine MRI. MATERIALS AND METHODS: We retrospectively reviewed 69 pediatric non-sedated fast spine MRI exams performed in 57 patients. Two blinded readers provided image quality ratings for the evaluation of bones, cranio-cervical junction, cerebrospinal fluid (CSF) spaces, spinal cord, soft tissues, ligaments, and overall diagnostic quality on a 1-5 scale, and determined whether there was evidence of syringomyelia, abnormal conus medullaris position, or filum terminale abnormality. RESULTS: Mean patient age was 7.2 years (age range ≤ 1-17). Indications included syringomyelia (n=25), spinal dysraphism (n=4), combination of both syringomyelia and spinal dysraphism (n=8), and other miscellaneous indications (n=32). The inter-observer agreement ranged between moderate and very good for each variable (Cohen's weighted kappa] range=0.45-0.69). The highest image quality ratings were given to CSF spaces (mean image quality=3.5/5 ± 0.8) and cranio-cervical junction evaluations (3.5/5 ± 0.9). Overall diagnostic quality was worst in the <5 years group (P=0.006). Readers independently identified a cervical spinal cord syrinx in 6 cases, and 1 mm spinal cord central canal dilation in one case. Readers agreed on the position of the conus medullaris in 92% of cases (23/25 cases). CONCLUSION: Non-sedated pediatric spine MRI can be an effective diagnostic test to evaluate for spine pathology, especially syringomyelia, Chiari malformation, and conus medullaris anatomy.

Use of a Prophylactic Retrograde-Flushing Device in High-Risk Pediatric Patients with Ventriculoperitoneal Shunts: A Technical Note.

INTRODUCTION: Ventriculoperitoneal shunt (VPS) malfunction rates are as high as 40% in the first year with posthemorrhagic hydrocephalus (PHH) patients having the highest proximal occlusion risk. Debris, protein, and cellular ingrowth most commonly obstruct the proximal ventricular catheter and/or valve. Historically, no preventative methods have demonstrated efficacy. We present a technical note and case series describing the use of a retrograde proximal flushing device and prophylactic flushing protocol to maintain ventricular catheter patency and reduce proximal shunt occlusions. METHODS: We present our 2.8-4-year follow-up data on the first 9 pediatric cases of ReFlow (Anuncia Inc, Scottsdale, AZ) device implantation combined with routine prophylactic flushing. Rationale for device implantation, patient selection, surgical procedure details, postoperative follow-up, and prophylactic flushing protocol are discussed as well as pre- and postimplantation ventricular catheter obstruction rates. We include a technical note on the device setup and prophylactic flushing protocol. RESULTS: Patient average age was 5.6 years and all patients had PHH. Minimal follow-up was 2.8 years (range 2.8-4 years). Prophylactic flushing was initiated between 2 and 14 days after ReFlow implantation and has continued as of the last follow-up. In 7 patients, ReFlow implantation occurred during the revision of an existing shunt and in two, implantation was coincident with initial VPS placement. In the 2 years preceding ReFlow and prophylactic flushing, 14 proximal shunt failures occurred in the 7 patients with existing VPS. This was reduced to only one proximal shunt failure in all 9 patients during the full follow-up period after ReFlow and prophylactic flushing. CONCLUSION: Pediatric VPS placement carries high rates of proximal catheter occlusion, often leading to emergency surgery, morbidity, or even death. The ReFlow device along with routine prophylactic flushing may reduce proximal obstruction and need for revision surgery. Higher patient numbers and longer follow-up periods are necessary to further elucidate the safety and effect of such a device on longer term shunt failures and revision surgery.

Complement Drives Chronic Inflammation and Progressive Hydrocephalus in Murine Neonatal Germinal Matrix Hemorrhage.

Germinal matrix hemorrhage (GMH) is a pathology that occurs in infancy, with often devastating long-term consequences. Posthemorrhagic hydrocephalus (PHH) can develop acutely, while periventricular leukomalacia (PVL) is a chronic sequala. There are no pharmacological therapies to treat PHH and PVL. We investigated different aspects of the complement pathway in acute and chronic outcomes after murine neonatal GMH induced at postnatal day 4 (P4). Following GMH-induction, the cytolytic complement membrane attack complex (MAC) colocalized with infiltrating red blood cells (RBCs) acutely but not in animals treated with the complement inhibitor CR2-Crry. Acute MAC deposition on RBCs was associated with heme oxygenase-1 expression and heme and iron deposition, which was reduced with CR2-Crry treatment. Complement inhibition also reduced hydrocephalus and improved survival. Following GMH, there were structural alterations in specific brain regions linked to motor and cognitive functions, and these changes were ameliorated by CR2-Crry, as measured at various timepoints through P90. Astrocytosis was reduced in CR2-Crry-treated animals at chronic, but not acute, timepoints. At P90, myelin basic protein and LAMP-1 colocalized, indicating chronic ongoing phagocytosis of white matter, which was reduced by CR2-Crry treatment. Data indicate acute MAC-mediated iron-related toxicity and inflammation exacerbated the chronic effects of GMH.

Pediatric Cranioplasty Using Hydroxyapatite Cement: A Retrospective Review and Preliminary Computational Model.

INTRODUCTION: Cranioplasty is a standard technique for skull defect repair. Restoration of cranial defects is imperative for brain protection and allowing for homeostasis of cerebral spinal fluid within the cranial vault. Calcium phosphate hydroxyapatite (HA) is a synthetic-organic material that is commonly used in cranioplasty. We evaluate a patient series undergoing HA cement cranioplasty with underlying bioresorbable mesh for various cranial defects and propose a preliminary computational model for understanding skull osteointegration. METHODS: A retrospective review was performed at the institution for all pediatric patients who underwent HA cement cranioplasty. Seventeen patients were identified, and success of cranioplasty was determined based on clinical and radiographic follow-up. A preliminary computational model was developed using bone growth and scaffold decay equations from previously published literature. The model was dependent on defect size and shape. Patient data were used to optimize the computational model. RESULTS: Seventeen patients were identified with an average age of 6 ± 5.6 years. Average defect size was 11.7 ± 16.8 cm2. Average time to last follow-up computer tomography scan was 10 ± 6 months. Three patients had failure of cranioplasty, all with a defect size above 15 cm2. The computational model developed shows a constant decay rate of the scaffold, regardless of size or shape. The bone growth rate was dependent on the shape and number of edges within the defect. Thus, a star-shaped defect obtained a higher rate of growth than a circular defect because of faster growth rates at the edges. The computational simulations suggest that shape and size of defects may alter success of osteointegration. CONCLUSION: Pediatric cranioplasty is a necessary procedure for cranial defects with a relatively higher rate of failure than adults. Here, we use HA cement to perform the procedure while creating a preliminary computational model to understand osteointegration. Based on the findings, cranioplasty shape may alter the rate of integration and lead to higher success rates.

A Role of Complement in the Pathogenic Sequelae of Mouse Neonatal Germinal Matrix Hemorrhage.

Germinal matrix hemorrhage (GMH) is a devastating disease of infancy that results in intraventricular hemorrhage, post-hemorrhagic hydrocephalus (PHH), periventricular leukomalacia, and neurocognitive deficits. There are no curative treatments and limited surgical options. We developed and characterized a mouse model of GMH based on the injection of collagenase into the subventricular zone of post-natal pups and utilized the model to investigate the role of complement in PHH development. The site-targeted complement inhibitor CR2Crry, which binds deposited C3 complement activation products, localized specifically in the brain following its systemic administration after GMH. Compared to vehicle, CR2Crry treatment reduced PHH and lesion size, which was accompanied by decreased perilesional complement deposition, decreased astrocytosis and microgliosis, and the preservation of dendritic and neuronal density. Complement inhibition also improved survival and weight gain, and it improved motor performance and cognitive outcomes measured in adolescence. The progression to PHH, neuronal loss, and associated behavioral deficits was linked to the microglial phagocytosis of complement opsonized neurons, which was reversed with CR2Crry treatment. Thus, complement plays an important role in the pathological sequelae of GMH, and complement inhibition represents a novel therapeutic approach to reduce the disease progression of a condition for which there is currently no treatment outside of surgical intervention.

Primary Central Nervous System Posttransplant Lymphoproliferative Disorder After Kidney Transplant.

Primary central nervous system posttransplant lymphoproliferative disorder is a rare complication of solid organ transplantation, with increasing incidence in children given a steady rise in pediatric solid organ transplants. Given similar imaging appearance to many opportunistic infections, a high degree of awareness is required for prompt and early diagnosis. We report a case of primary central nervous system posttransplant lymphoproliferative disorder presenting as a single rim enhancing lesion with central restricted diffusion mimicking an intracranial abscess.

Fast Brain Magnetic Resonance Imaging With Half-Fourier Acquisition With Single-Shot Turbo Spin Echo Sequence in Detection of Intracranial Hemorrhage and Skull Fracture in General Pediatric Patients: Preliminary Results.

OBJECTIVE: The objective of this study was to determine the accuracy of fast brain magnetic resonance imaging (MRI) in the detection of intra- and extra-axial intracranial hemorrhage compared with standard-of-care computed tomography (CT) or MRI in pediatric patients. Unlike previous studies, we did not focus exclusively on patients with head trauma. We evaluated the fast brain MRI findings in a general pediatric population referred for indications other than evaluation of ventricular size. METHODS: We retrospectively reviewed 48 pediatric patients with indications other than hydrocephalus and shunt follow-up, who underwent a standard head CT or standard MRI within 15 days of the fast brain MRI. All fast brain MRI scans included half-Fourier acquisition with single-shot turbo spin echo (HASTE) sequences in the axial, coronal, and sagittal plane. Two neuroradiologists blinded to patient information and study indications reviewed the fast brain MRI studies independently and then concurrently. RESULTS: A total of 48 patients met the inclusion and exclusion criteria. The median and mean time interval between the standard and fast imaging were 2 and 3.9 days, respectively. The sensitivity and specificity of fast brain MRI to detect intraparenchymal hemorrhage were 100% and 97%, respectively. The sensitivity and specificity of fast brain MRI in the detection of extra-axial hemorrhage (subdural and/or epidural) were 86% and 96%, respectively. The sensitivity and specificity of fast brain MRI were, respectively, 10% and 100% for subarachnoid hemorrhage, 50% and 100% for intraventricular hemorrhage, and 47% and 97% for skull fracture, respectively. CONCLUSIONS: Our results show that fast brain MRI with HASTE sequence is as sensitive as CT and standard MRI in the detection of intra-axial hemorrhage and has moderate sensitivity in the detection of extra-axial hemorrhage. Our preliminary results show that T2-weighted HASTE imaging may be suitable for the follow-up of intraparenchymal and extra-axial (subdural and/or epidural) hemorrhages.

Serum level of Vitamin D is associated with COVID-19 mortality rate in hospitalized patients.

BACKGROUND: Due to widespread of coronavirus disease 2019 (COVID-19) infection, identification of its risk factors and clinical characteristics are important. The aim of the present study was to assess Vitamin D levels in individuals with severe acute respiratory syndrome coronavirus-19 infection and to report on its potential as a predictive marker. MATERIALS AND METHODS: All patients, diagnosed with COVID-19 infection from February 16 to March 21, 2020, and referred to Firoozgar Hospital, Tehran, Iran, were enrolled in this study. Vitamin D analysis was undertaken on patient serum samples using a commercial kit (Pars Azmoon Co., Tehran, Iran). SPSS v. 22 was used for statistical analysis. RESULTS: Vitamin D serum concentration was analyzed in a total of 317 patients whose mean age ± standard deviation was 62.05 ± 15 years and with 62.5% being male. A significant association of Vitamin D level and death was observed. Higher levels of serum Vitamin D had protection against death (odds ratio = 0.955 [95% confidence interval = 0.923-0.988], P = 0.008). CONCLUSION: As a preliminary study in the Iranian population who suffered COVID-19 disease, we identified that Vitamin D deficiency was associated with a higher death rate and intensive care unit admission.

Recurrent Stroke in a Child With Atlantoaxial Instability Following Chiropractic Manipulation.

Pediatric stroke presents with a variety of signs and symptoms. Correct modality of imaging is essential in decreasing the time from symptom onset to appropriate management. Evaluation of pediatric stroke should include both blood work as well as imaging in a parallel rather than a sequential matter. We report a case of a child with a bow hunter's stroke that was challenging to diagnose. This type of stroke happens when the vertebral artery is occluded at the atlantoaxial or subaxial level during neck rotation. This case demonstrates that workup of stroke should be comprehensive to include all mechanical and anatomic possibilities before investigating rarer hypercoagulable disorders.

Rathke's Cleft Cyst Apoplexy in Two Teenage Sisters.

Rathke's cleft cysts (RCC) are sellar-suprasellar cysts that are usually discovered incidentally given their indolent clinical course. When symptoms do arise, the most common clinical presentation is headache, visual field deficits due to visual pathway compression, diplopia due to cavernous sinus compression, chemical meningitis due to spillage of the cyst contents, endocrine dysfunction, and very rarely apoplexy. We present 2 cases of RCC in sisters who developed a sudden onset of symptoms in a manner similar to pituitary apoplexy. Interestingly, one of them had a very unusual presentation with seizure. We hypothesize that acute symptoms occur due to aggressive intracystic overproduction of mucopolysaccharides (with or without hemorrhage) and a resulting compressive syndrome or local irritation of surrounding structures by spillage of the cyst contents. RCC can be encountered incidentally in family members or may have a familiar predisposition. Since both sisters presented here developed apoplexy symptoms, we propose a more frequent follow-up with sequential imaging in patients with a family history of RCC. Transsphenoidal surgery with evacuation of the cyst contents is the treatment modality of choice, with excellent outcomes.

Immunological low-dose radiation modulates the pediatric medulloblastoma antigens and enhances antibody-dependent cellular cytotoxicity.

BACKGROUND: Immunotherapy can be an effective treatment for pediatric medulloblastoma (MB) patients. However, major subpopulations do not respond to immunotherapy, due to the lack of antigenic mutations or the immune-evasive properties of MB cells. Clinical observations suggest that radiation therapy (RT) may expand the therapeutic reach of immunotherapy. The aim of the present investigation is to study the effect of low-dose X-ray radiation (LDXR, 1 Gy) on the functional immunological responses of MB cells (DAOY, D283, and D341). METHODS: Induction of MB cell death was examined using the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assay. Production of reactive oxygen species (ROS) was measured by fluorescent probes. Changes in the expression of  human leukocyte antigen (HLA) molecules and caspase-3 activities during treatment were analyzed using Western blotting and caspase-3 assay. RESULTS: Western blot analysis demonstrated that LDXR upregulated the expression of HLA class I and HLA II molecules by more than 20% compared with control and high-dose (12 Gy) groups in vitro. Several of these HLA subtypes, such as MAGE C1, CD137, and ICAM-1, have demonstrated upregulation. In addition, LDXR increases ROS production in association with phosphorylation of NF-κB and cell surface expression of mAb target molecules (HER2 and VEGF). These data suggest that a combined LDXR and mAb therapy can create a synergistic effect in vitro. CONCLUSION: These results suggest that LDXR modulates HLA molecules, leading to alterations in T-cell/tumor-cell interaction and enhancement of T-cell-mediated MB cell death. Also, low-dose radiotherapy combined with monoclonal antibody therapy may one day augment the standard treatment for MB, but more investigation is needed to prove its utility as a new therapeutic combination for MB patients.

Mapping the neuropsychological profile of temporal lobe epilepsy using cognitive network topology and graph theory.

OBJECTIVE: Normal cognitive function is defined by harmonious interaction among multiple neuropsychological domains. Epilepsy has a disruptive effect on cognition, but how diverse cognitive abilities differentially interact with one another compared with healthy controls (HC) is unclear. This study used graph theory to analyze the community structure of cognitive networks in adults with temporal lobe epilepsy (TLE) compared with that in HC. METHODS: Neuropsychological assessment was performed in 100 patients with TLE and 82 HC. For each group, an adjacency matrix was constructed representing pair-wise correlation coefficients between raw scores obtained in each possible test combination. For each cognitive network, each node corresponded to a cognitive test; each link corresponded to the correlation coefficient between tests. Global network structure, community structure, and node-wise graph theory properties were qualitatively assessed. RESULTS: The community structure in patients with TLE was composed of fewer, larger, more mixed modules, characterizing three main modules representing close relationships between the following: 1) aspects of executive function (EF), verbal and visual memory, 2) speed and fluency, and 3) speed, EF, perception, language, intelligence, and nonverbal memory. Conversely, controls exhibited a relative division between cognitive functions, segregating into more numerous, smaller modules consisting of the following: 1) verbal memory, 2) language, perception, and intelligence, 3) speed and fluency, and 4) visual memory and EF. Overall node-wise clustering coefficient and efficiency were increased in TLE. SIGNIFICANCE: Adults with TLE demonstrate a less clear and poorly structured segregation between multiple cognitive domains. This panorama suggests a higher degree of interdependency across multiple cognitive domains in TLE, possibly indicating compensatory mechanisms to overcome functional impairments.

Pediatric Intraparenchymal Meningioma: Case Report and Comparative Review.

Intra-axial (intraparenchymal) meningiomas are an extremely rare pathology with only dozens of cases reported. In children, the unusual characteristics of intraparenchymal meningiomas can easily create an atypical preoperative differential. The authors present the case of an otherwise healthy 14-year-old girl presenting with new-onset seizures and an intracranial lesion upon investigation. CT and MRI revealed a lesion both cystic and calcified in nature, residing completely within the brain parenchyma. Upon operative examination, the mass was found to be completely surrounded by cortex and to be of two disparate consistencies. Pathological analysis revealed the mass to be a meningioma. Despite the rarity in children, meningiomas must be kept on the differential for cystic, calcified lesions. The differential diagnosis based on imaging as well as the operative strategy taken are further discussed. In addition, the authors review the current understanding of pediatric meningiomas as compared with adult meningiomas.

Differential vulnerability of white matter structures to experimental infantile hydrocephalus detected by diffusion tensor imaging.

PURPOSE: The differential vulnerability of white matter (WM) to acute and chronic infantile hydrocephalus and the related effects of early and late reservoir treatment are unknown, but diffusion tensor imaging (DTI) could provide this information. Thus, we characterized WM integrity using DTI in a clinically relevant model. METHODS: Obstructive hydrocephalus was induced in 2-week-old felines by intracisternal kaolin injection. Ventricular reservoirs were placed 1 (early) or 2 (late) weeks post-kaolin and tapped frequently based solely on neurological deficit. Hydrocephalic and age-matched control animals were sacrificed 12 weeks postreservoir. WM integrity was evaluated in the optic system, corpus callosum, and internal capsule prereservoir and every 3 weeks using DTI. Analyses were grouped as acute (<6 weeks) or chronic (≥6 weeks). RESULTS: In the corpus callosum during acute stages, fractional anisotropy (FA) decreased significantly with early and late reservoir placement (p = 0.0008 and 0.0008, respectively), and diffusivity increased significantly in early (axial, radial, and mean diffusivity, p = 0.0026, 0.0012, and 0.0002, respectively) and late (radial and mean diffusivity, p = 0.01 and 0.0038, respectively) groups. Chronically, the corpus callosum was thinned and not detectable by DTI. FA was significantly lower in the optic chiasm and tracts (p = 0.0496 and 0.0052, respectively) with late but not early reservoir placement. In the internal capsule, FA in both reservoir groups increased significantly with age (p < 0.05) but diffusivity remained unchanged. CONCLUSIONS: All hydrocephalic animals treated with intermittent ventricular reservoir tapping demonstrated progressive ventriculomegaly. Both reservoir groups demonstrated WM integrity loss, with the CC the most vulnerable and the optic system the most resilient.

Utility of intracranial pressure monitoring as a diagnostic tool in pediatric ventriculomegaly.

OBJECTIVE: Intracranial pressure (ICP) monitoring is commonly utilized for identifying pathologic ICP in cases of traumatic brain injury; however, its utility in hydrocephalic children has not been elucidated. Although patients with typical (pressure-active) hydrocephalus present with clear signs and/or symptoms and the need for cerebrospinal fluid (CSF) diversion is often clear, others may have arrested or pressure-compensated hydrocephalus with pathologic ICP elevation masked by ambiguous signs or are completely asymptomatic. Without treatment these pathologic ICP elevations may affect neurologic development or crescendo over time leading to neurological decline. The purpose of this study is to investigate the utility of ICP monitoring as a diagnostic tool in this relatively common patient population and identify ventriculomegaly patients with and without pathologic ICP, thus improving accuracy of identifying those with and without surgical needs. METHODS: 36 patients (≤ 17 years old) underwent 41 inpatient ICP recording sessions between 2016 and 2022 and were retrospectively reviewed. This included patients with a history of severe, nonprogressive ventriculomegaly and normal fundoscopic examinations lacking traditional signs and symptoms concerning for elevated ICP. Nighttime pathological plateau waves were defined as sustained elevations of ICP ≥ 2x baseline for a duration of ≥ 5 minutes. RESULTS: The mean age of patients was 5.5 years old (range 0-17 years old). 46.3% of patients had prior endoscopic third ventriculostomy (ETV), 14.6% had prior ventriculoperitoneal shunt (VPS), and 39% were without prior surgical intervention. Roughly half (51.2%) of patients had congenital ventriculomegaly while other patients had ventriculomegaly due to other pathologies such as germinal matrix hemorrhage/intraventricular hemorrhage (GMH/IVH) (29.3%), stroke (4.9%), cerebral infections/meningitis (2.4%), or unknown etiology (12.2%). The average procedure time was 19.1 ± 10.5 minutes, and mean length of stay was 2.8 ± 0.7 days. Pathologic ICP was demonstrated in 12 cases (29.3%), 4 (33.3%) of which were asymptomatic. Pathologic ICP was found in 7 of 19 (36.8%) in the prior ETV group, 1 of 6 (16.7%) in prior shunt group, and 4 of 16 (25%) in the non-surgical group (p = 0.649). Among those with pathologic ICP, 6 (50%) cases received an ETV, 5 (41.7%) cases underwent VPS placement, and 1 (8.3%) case underwent a VPS revision. There were no infectious complications or cases of hemorrhage. 4 patients required repositioning of the ICP monitor due to dislodgement. CONCLUSION: Inpatient ICP monitoring is a safe and effective diagnostic tool for evaluating the presence of pathologic ICP in severe, persistent non-progressive ventriculomegaly. The use of ICP monitoring may aid in identifying patients with pressure-compensated hydrocephalus who demonstrate pathologic ICP where surgical intervention may be warranted, while preventing unnecessary CSF diversion in those without pathology.

Exoscope Efficacy and Feasibility in Pediatric Spinal Neurosurgery: A Single-Institution Cohort Case Series.

BACKGROUND: The exoscope has emerged as an efficacious microscope in adult spinal neurosurgery providing improved operative field visibility and surgeon ergonomics. However, outcome data and feasibility are underrepresented in the pediatric literature. We present the largest case series aimed at assessing operative and clinical outcomes in pediatric patients undergoing various exoscope-assisted spinal surgeries. METHODS: A retrospective review was conducted on all consecutive pediatric (age <18 years) spinal surgeries performed with the use of an exoscope by 3 senior surgeons at a single institution from 2020-2023. Demographics and clinical and operative outcomes were reviewed and analyzed. RESULTS: Ninety-six exoscope-assisted pediatric spine surgeries were performed on 89 unique patients, 41 (42.7%) of which were male. The mean age at surgery was 12 (±5.3) years. Spinal cord detethering (55.8%) was the most common procedure performed. The overall mean operative time for all procedures was 155 (±86) minutes, and the mean estimated blood loss was 18 (±41) mL. The mean length of stay was 5.4 (±6.5) days. There were 14 (14.6%) patients with complications in this cohort. At final follow-up, 64 (83.1%) of symptomatic patients reported neurologic symptom improvement. CONCLUSIONS: Using the exoscope in a variety of pediatric spinal surgeries resulted in an acceptable average operative time, estimated blood loss, length of stay, and rate of neurologic symptom improvement. The exoscope appears to be an efficacious option for pediatric neurosurgical spinal procedures.

The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Oberlin transfer and partial radial to axillary nerve neurotization to repair an explosive traumatic injury to the brachial plexus in a child: case report.

PURPOSE: Explosive injuries to the pediatric brachial plexus are exceedingly rare and as such are poorly characterized in the medical literature. METHODS: Herein, we describe an 8-year-old who was struck in the neck by a piece of shrapnel and suffered multiple vascular injuries in addition to a suspected avulsion of the cervical 5 and 6 ventral rami. The patient had a complete upper brachial plexus palsy and failed to demonstrate any clinical improvement at 6-months follow-up. He was taken to the operating from for a partial ulnar to musculocutaneous nerve neurotization as well as a partial radial to axillary nerve neurotization. RESULTS: The patient's motor exam improved from a Medical Research Council scale 1 to 4+ for biceps brachii and 0 to 4 deltoid function with greater than 90° of shoulder abduction. CONCLUSIONS: This outcome supports complex neurotization techniques as viable treatment options for persistent motor deficits following an upper brachial plexus injury in older, non-infant age, children.