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.

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.

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.

A Multicenter Study Investigating the Surgeon Experience with a Robotic-Assisted Exoscope as Part of the Neurosurgical Armamentarium.

BACKGROUND: Improvement of visualization tools in neurosurgery such as the exoscope has raised the question of how this technology compares to the conventional microscope for surgeon ergonomics, discomfort, and patient outcomes. Exoscopes have the advantage of greater optical zoom, resolution, and illumination at a lower light intensity. Heads-up display for both the primary surgeon and other assistants permits neutral positioning of the surgeons while placing the camera in more angled positions. In a survey sample, this study assesses the surgeon experience utilizing 3D exoscope in general neurosurgery cases. METHODS: Data weere recorded by 8 surgeons at 5 separate hospitals utilizing a mobile phone application survey. Surgeons recorded information about case type, intraoperative clinical outcomes such as blood loss and extent of resection, whether fluorescence visualization was used, as well as surgeon pain when compared to matched cases using conventional tools. RESULTS: A total of 155 neurosurgical cases were recorded in this multisite study, including 72% cranial cases and 28% spinal cases. Of the cranial cases, 76% were brain tumor resections (31% of which were brain metastases). Surgeons reported significantly less neck (P < 0.0001) and back (P < 0.0001) pain in cases when using the robotic exoscope compared with the conventional microscope or surgical loupes. Surgeons did not convert to a microscope in any case. CONCLUSIONS: The exoscope provides excellent delineation of tissue with high resolution. Surgeon pain was markedly reduced with the robotic exoscope when compared with conventional technology, which may reduce work-related injury and fatigue, potentially leading to better patient outcomes.

Giant Pediatric Dural Arteriovenous Fistula Management Using a Combined Operative Approach in a Hybrid Neuroendovascular Surgery Suite: Technical Nuances and Review of Literature.

BACKGROUND: Pediatric intracranial dural arteriovenous fistulas (dAVFs) are rare, complex entities usually presenting with macrocephaly from increased intracranial pressures at a young age. In the setting of a symptomatic intracranial dAVF that has undergone multiple endovascular treatments with subsequent recurrence or failed embolization attempts, the intracranial venous system can become inaccessible by traditional transvenous and transarterial routes. Direct puncture of the venous sinus for endovascular access after surgical exposure is a viable option. OBJECTIVE: To describe the technical nuances and available literature for direct puncture of the venous sinus for endovascular access in a pediatric patient with dAVF. METHODS: The clinical characteristics were reviewed and reported for a patient who underwent direct puncture of the venous sinus for endovascular access. In addition, a literature review was conducted for relevant literature pertaining to this technique and its associated indications, outcomes, and complications. RESULTS: Only 2 other reports of direct puncture of venous sinus for endovascular access after surgical exposure were found in the literature. Our patient achieved a favorable outcome with complete dAVF obliteration. CONCLUSION: Direct puncture of the venous sinus for endovascular access after surgical exposure for complex dAVFs that are inaccessible by transvenous or transarterial routes is a practical and safe approach to intracranial venous access that should be part of the vascular neurosurgeon's arsenal.

3-Dimensional Exoscope for Far Lateral Approach to Pontomedullary Cavernous Malformation.

Brainstem cavernous malformations account for 15%-18% of all central nervous system cavernomas and are histologically characterized by thin-walled, low-pressure capillaries, classically without intervening brain tissue.1,2 Cavernomas may be sporadic, typically characterized by a single lesion, or inherited. The inherited form is most often autosomal dominant with incomplete penetrance and variable expression. Multiple cavernomas are associated with the familial form; although this is not always the case, genetic workup should be pursued.3,4 Clinical presentation typically includes focal neurologic deficit related to hemorrhage location, seizures, and rarely obstructive hydrocephalus.1,2 Indications for surgical management include severe or progressive neurologic dysfunction, lesion size ≥2 cm, recurring hemorrhages, and/or significant mass effect.5 Microsurgical resection of a cavernoma is associated with an overall 28% complication rate and perioperative neurologic morbidity upwards of 45% according to some series. Long-term surgical outcomes at 12 months are more reassuring: 84% reported their condition to have improved or remained the same, and the long-term morbidity rate is 14%.1,6 The location of the lesion dictates the approaches available-cavernomas in the pons or medulla are commonly approached via a retrosigmoid or retrolabyrinthine approach, while more ventral pathologies in this region necessitate a far lateral approach.1,5,7,8 In Videos 1 and 2, we describe our experience with an exoscope-assisted far lateral approach to a pontomedullary cavernoma in a 10-year-old male presenting with numerous cavernomas and confirmed gene mutation. We demonstrate the exoscope's unparalleled visualization of the anterolateral brainstem, with nominal condylar drilling. The patient and his parents consented to the procedure and publication.

Complications and outcomes of posterior fossa decompression with duraplasty versus without duraplasty for pediatric patients with Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). METHODS: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. RESULTS: A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. CONCLUSIONS: PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

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.

Dural augmentation approaches and complication rates after posterior fossa decompression for Chiari I malformation and syringomyelia: a Park-Reeves Syringomyelia Research Consortium study.

OBJECTIVE: Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM. METHODS: The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft. RESULTS: A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain. CONCLUSIONS: In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.

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.

Juvenile xanthogranuloma of the subdural spaces mimicking chronic hematoma with positive FDG uptake on PET: case report.

The authors report an unusual presentation of juvenile xanthogranuloma (JXG), a non-Langerhans cell histiocytosis of infancy and early childhood. This entity typically presents as a cutaneous head or neck nodule but can manifest with more systemic involvement including in the central nervous system. However, currently there is limited information regarding specific imaging features differentiating JXG from other neuropathological entities, with diagnosis typically made only after tissue sampling. The authors reviewed the initial images of a young patient with shunt-treated hydrocephalus and enlarging, chronic, extraaxial processes presumed to reflect subdural collections from overshunting, and they examine the operative discovery of a mass lesion that was pathologically proven to be JXG. Their results incorporate the important associated histological and advanced imaging features, including previously unreported metabolic activity on FDG PET. Ultimately, the case underscores the need to consider JXG in differential diagnoses of pediatric intracranial masses and highlights the potential role of PET in the initial diagnosis and response to treatment.

Cystic angiomatosis skull lesion obliteration with neuroendovascular sclerotherapy as a unique treatment: case report.

Cystic angiomatosis is a rare bone condition with complex presentation and difficult treatment. Current management strategies have poorly tolerated side effects and a low likelihood of disease eradication. The control of calvarial lesions that are symptomatic usually involves surgical excision and subsequent cranioplasty. This paradigm can present with a risk of morbidity and mortality depending on the anatomy of the lesion. Here, the authors present a novel approach to a difficult-to-treat occipital calvarial lesion directly overlying the transverse sinus, performing a small, partial-thickness craniectomy and alcohol sclerotherapy in a combined neurosurgery-neuroendovascular approach. At 3 years after treatment, the authors noted a complete, encouraging radiographic and clinical outcome.

Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established. METHODS: Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length. RESULTS: The authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16-0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34-1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12-0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02-2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31-3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67-5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate. CONCLUSIONS: These data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.

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.

Pediatric Cranial Fasciitis: Discussion of Cases and Systematic Review of the Literature.

OBJECTIVE: Cranial fasciitis is a rare benign mass that typically presents in pediatric patients from 3 weeks to 6 years of age. It is classified as a subset of nodular fasciitis and was first reported in 1980. This study evaluates the literature for common characteristics that may affect diagnosis and treamtent. METHODS: We describe the case of a 13-month-old girl with a history of accidental head trauma 7 months before presentation and the case of a 5-month-old girl with an expansile skull lesion. We also performed a systematic review of the reported data on cranial fasciitis, including a total of 57 reported studies with 80 unique cases. RESULTS: There were 80 total cases reviewed in the literature. There was a male predominence, 1.75:1. The average age at presentation was 5.2 years. The most common causes for this lesion were idiopathic (65%), blunt trauma (14%) and radiation therapy (7%). Overall, there was a 9% recurrence rate following treatment. CONCLUSION: We report the characteristics at presentation, including, to the best of our knowledge, the first account of gender differences, and the treatment modalities used in the included studies and the implications in relation to the recurrence rates.

A novel technology to model pressure-induced cellular injuries in the brain.

BACKGROUND: Elevated intracranial pressure (ICP) accompanying a number of neurological emergencies is poorly understood, and lacks a model to determine cellular pathophysiology. This limits our ability to identify cellular and molecular biomarkers associated with the pathological progression from physiologic to pathologic ICP. NEW METHOD: We developed an ex vivo model of pressure-induced brain injury, which combines 3D neural cell cultures and a newly developed Pressure Controlled Cell Culture Incubator (PC3I). Human astrocytes and neurons maintained in 3D peptide-conjugated alginate hydrogels were subjected to pressures that mimic both physiologic and pathologic levels of ICP for up to 48h to evaluate the earliest impacts of isolated pressure on cellular viability and quantify early indicators of pressure-induced cellular injury. RESULTS: Compared to control cell cultures grown under physiologic pressure, sustained pathologic pressure exposure increased the release of intracellular ATP in a cell-specific manner. Eighteen hours of sustained pressure resulted in increased ATP release from neurons but not astrocytes. COMPARISON WITH EXISTING METHODS: Cell culture incubators maintain cultures at normal atmospheric pressure. Based on multiple literature searches, we are not aware of any other cell culture incubator systems that modify the pressure at which primary CNS cells are maintained. CONCLUSION: This model simulates the clinical features of elevated ICP encountered in patients with hydrocephalus, and provides a first estimate of the pathological signaling encountered during the earliest perid of progression in neonatal hydrocephalus. This model should provide a means to better understand the pathological biomarkers associated with the earliest stages of elevated ICP.

Transgenerational Inheritance of Familial Lipomyelomeningocele.

Lipomyelomeningocele is a type of neural tube defect characterized by lipomatous tissue causing a defect in the vertebrae, infiltrating the dura, and tethering the spinal cord. Despite significant neurologic consequences, the underlying etiology remains poorly understood. We present a father and son with remarkably similar presentations of lipomyelomeningocele. Genetic testing did not reveal an underlying cause but whole exome sequencing identified variants in the ARHGAP29 and RADIL genes in the proband and his affected father. Genetic analyses of asymptomatic family members revealed several carriers of the ARHGAP29 or RADIL variants, but only the proband and his father carried both variants, suggesting a possible shared genetic mechanism. Rare cases of siblings affected with lipomyelomeningocele have suggested the possibility of autosomal recessive or germline mosaicism. We present the first documented cases of transgenerational lipomyelomeningocele with important implications for family counseling about the recurrence of lipomyelomeningocele.

Basilar artery occlusion in a child treated successfully with mechanical thrombectomy using ADAPT.

Acute ischemic stroke (AIS) in the pediatric population is rare. Furthermore, it is common for physicians to take significantly longer diagnosing a posterior circulation stroke in a child than in an adult. There are increasing case reports in the literature of treating AIS in children with intravenous tissue plasminogen activator, intra-arterial thrombolysis, and/or mechanical thrombectomy. We present the first case of pediatric AIS treated using a direct aspiration first pass technique (ADAPT) as a means of mechanical thrombectomy.