Induction of Interleukin-9-Producing Mucosal Mast Cells Promotes Susceptibility to IgE-Mediated Experimental Food Allergy.

Experimental IgE-mediated food allergy depends on intestinal anaphylaxis driven by interleukin-9 (IL-9). However, the primary cellular source of IL-9 and the mechanisms underlying the susceptibility to food-induced intestinal anaphylaxis remain unclear. Herein, we have reported the identification of multifunctional IL-9-producing mucosal mast cells (MMC9s) that can secrete prodigious amounts of IL-9 and IL-13 in response to IL-33, and mast cell protease-1 (MCPt-1) in response to antigen and IgE complex crosslinking, respectively. Repeated intragastric antigen challenge induced MMC9 development that required T cells, IL-4, and STAT6 transcription factor, but not IL-9 signals. Mice ablated of MMC9 induction failed to develop intestinal mastocytosis, which resulted in decreased food allergy symptoms that could be restored by adoptively transferred MMC9s. Finally, atopic patients that developed food allergy displayed increased intestinal expression of Il9- and MC-specific transcripts. Thus, the induction of MMC9s is a pivotal step to acquire the susceptibility to IgE-mediated food allergy.

MN1 altered astroblastoma with APC and LRP1B gene mutations: a unique variant in the cervical spine of a pediatric patient.

PURPOSE: Astroblastomas (AB) are high-grade neoplasms which typically occur within the cerebral hemisphere. However, given the rarity of this neoplasm and the number of variants, the relevance of this molecular makeup is unknown. We sought to describe the clinical presentation, treatment, and pathological analysis of a novel MN1 (meningioma 1) cervical spinal cord astroblastoma variant presenting in a pediatric patient. METHODS: A retrospective review of electronic medical records was performed with an emphasis on neuroimaging, perioperative course, and pathological analysis. RESULTS: An 11-month-old male with no significant history presented with two weeks of neck stiffness and cervicalgia. Neurologically, the patient was intact without signs of infection or trauma. Cervical CT was unremarkable. A subsequent MRI demonstrated a heterogeneously enhancing intramedullary lesion extending from the craniocervical junction to T4. The patient was treated with perioperative steroids and underwent C1-C3 laminectomies and C4-T4 laminotomies for tumor resection. Upon completion of the durotomy, an exophytic gray-red tumor was appreciated within the epidural space and gross total resection was achieved (no change on intraoperative neurophysiological monitoring) and confirmed on post-operative imaging. Immunohistochemical analysis was consistent with an astroblastoma with atypical diffuse positivity of CD56, CD99, and nuclear OLIG2. Molecular analysis revealed not only MN1 alterations but also changes in genes encoding APC and LRP1B. Both alterations were not previously documented to be associated with an astroblastoma. CONCLUSION: Our case represents the first report of an infant with an MN1 astroblastoma with APC and LRP1B gene alterations in the cervical spine. Gross total resection paired with a detailed histopathologic analysis is vital for optimizing adjuvant treatment.

The implications of antithrombotic agents on subdural hematoma evacuation: what does "reversal" truly entail?

OBJECTIVE: The optimal perioperative management of antithrombotic therapy (ATT) in patients requiring urgent neurosurgical intervention for subdural hematoma (SDH) is poorly understood. The delicate equilibrium of effective hemostasis while preventing thrombosis is complex and relies on numerous factors such as indication for and type of ATT, medical comorbidities, and extent of neurological injury. This study aimed to analyze the impact of ATT and reversal strategies on surgical outcomes to highlight current challenges in the management of these high-risk patients. METHODS: The authors performed a retrospective surgical cohort analysis of 100 patients undergoing urgent SDH evacuation at a level I trauma center between March 2020 and May 2021. The patients were first stratified into two cohorts based on preoperative ATT use and then further segregated by receipt of reversal agents. Statistical analysis included the chi-square test, Welch two-sample t-test, and multivariate logistic regression. The primary outcome was mortality. Secondary endpoints included radiographic SDH reexpansion, revision surgery, improvement in preoperative neurological deficits, and incidence of thromboembolism. A crossover cohort was secondarily analyzed in patients for whom ATT was interrupted for a minimum duration equal to effective drug metabolism. Finally, ATT reinitiation patterns were examined. RESULTS: Of 100 patients, 48% received ATT, 54.2% of whom were given reversal agents. ATT use was significantly associated with decreased rates of postoperative neurological improvement (p = 0.023) with trends toward increased mortality (p = 0.078), SDH reexpansion (p = 0.12), and need for revision surgery (p = 0.10). Patient crossover revealed a 4 times greater likelihood of death in patients without ATT interruption prior to surgery (p = 0.040) without an observable impact on secondary outcomes. ATT reversal contributed no improvement in outcomes other than a decreased intensive care unit length of stay when adjusted for in-hospital mortality (p = 0.014). The rate of postoperative thromboembolism following ATT reversal was 11.5%. ATT reinitiation was highly variable, occurring in 59.5% of patients, with median times of 17 and 15 days for antiplatelets and anticoagulants, respectively. CONCLUSIONS: Use of preoperative ATT portends poor clinical outcomes following nonelective SDH evacuation regardless of attempts to reverse these medications with replacement blood products. This study further reinforces the critical need for judicious use of ATT and optimization of reversal strategies in high-risk patient populations as best guided by multidisciplinary teams and evolving clinical practice guidelines.

Adapting Clinical Practice of Thrombolysis for Acute Ischemic Stroke Beyond 4.5 Hours: A Review of the Literature.

Several clinical trials have demonstrated that advanced neuroimaging can select patients for recanalization therapy in an extended time window. The favorable functional outcomes and safety profile of these studies have led to the incorporation of neuroimaging in endovascular treatment guidelines, and most recently, also extended to decision making on thrombolysis. Two randomized clinical trials have demonstrated that patients who are not amenable to endovascular thrombectomy within 4.5 hours from symptoms discovery or beyond 4.5 hours from the last-known-well time may also be safely treated with intravenous thrombolysis and have a clinical benefit above the risk of safety concerns. With the growing aging population, increased stroke incidence in the young, and the impact of evolving medical practice, healthcare and stroke systems of care need to adapt continuously to provide evidence-based care efficiently. Therefore, understanding and incorporating appropriate screening strategies is critical for the prompt recognition of potentially eligible patients for extended-window intravenous thrombolysis. Here we review the clinical trial evidence for thrombolysis for acute ischemic stroke in the extended time window and provide a review of new enrolling clinical trials that include thrombolysis intervention beyond the 4.5 hour window.

Depression and glioblastoma, complicated concomitant diseases: a systemic review of published literature.

Glioblastoma multiforme (GBM) is the most common primary brain cancer. Depression is a common co-morbidity of this condition. Despite this common interaction, relatively little research has been performed on the development of GBM-associated depression. We performed a literary search of the PubMed database for articles published relating to GBM and depression. A total of 85 articles were identified with 46 meeting inclusion criteria. Depression significantly impacts care, decreasing medication compliance, and patient survival. Diagnostically, because depression and GBM share intricate neuro-connectivity in a way that effect functionality, these diseases can be mistaken for alternative psychological or pathological disorders, complicating care. Therapeutically, anti-depressants have anti-tumor properties; yet, some have been shown to interfere with GBM treatment. One reason for this is that the pathophysiological development of depression and GBM share several pathways including altered regulation of the 5-HT receptor, norepinephrine, and 3':5'-cyclic monophosphate. Over time, depression can persist after GBM treatment, affecting patient quality of life. Together, depression and GBM are complicated concomitant diseases. Clinicians must be aware of their co-existence. Because of overlapping molecular pathways involved in both diseases, careful medication selection is imperative to avoid potential adverse interactions. Since GBMs are the most common primary brain cancer, physicians dealing with this disease should be prepared for the development of depression as a potential sequela of this condition, given the related pathophysiology and the known poor outcomes.

Outcomes of treatment modalities for ruptured intracranial aneurysms based on age - A meta-analysis.

BACKGROUND: Data comparing the effect of age on outcomes of patients who underwent either endovascular coiling (EVC) or neurosurgical clipping (NSC) for ruptured intracranial aneurysms remains limited. OBJECTIVE: To better elucidate the preferred intervention for ruptured aneurysm management by presenting the results of our systematic review of the literature that evaluated the potential advantages of the two interventions between different age groups. METHODS: Systematic review of PubMed and Embase was performed (2002 - June 10, 2019) following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2013 guidelines. Median ages of EVC and NSC cohorts were 54 and 56, respectively. Ages below the median were used in our "younger" cohort; ages above the median were used in our "older" cohort. RESULTS: We reviewed 13 studies on 7,137 patients. In the younger cohort, there were 2840 (EVC: 1412, NSC: 1428) patients. In the older cohort, there were 4297 (EVC: 2552, NSC: 1745) patients. Overall, there was a significant difference in functionality between EVC (77.70%) and NSC (69.23%) (OR=1.69; 95% C.I.: 1.10-2.60, p = 0.0212). In our younger cohort, functionality was significantly different between EVC (77%) and NSC (69%) (OR=1.54; 95% C.I.: 1.29-1.84, p < 0.001). For the older cohort, there was no significant difference in functionality, complications, or efficacy. CONCLUSIONS: We have highlighted the importance of considering age prior to deciding which intervention is most appropriate for ruptured aneurysms, with higher morbidity and mortality with NSC versus EVC in the younger population.

HAX-1 regulates SERCA2a oxidation and degradation.

Ischemia/reperfusion injury is associated with contractile dysfunction and increased cardiomyocyte death. Overexpression of the hematopoietic lineage substrate-1-associated protein X-1 (HAX-1) has been shown to protect from cellular injury but the function of endogenous HAX-1 remains obscure due to early lethality of the knockout mouse. Herein we generated a cardiac-specific and inducible HAX-1 deficient model, which uncovered an unexpected role of HAX-1 in regulation of sarco/endoplasmic reticulum Ca-ATPase (SERCA2a) in ischemia/reperfusion injury. Although ablation of HAX-1 in the adult heart elicited no morphological alterations under non-stress conditions, it diminished contractile recovery and increased infarct size upon ischemia/reperfusion injury. These detrimental effects were associated with increased loss of SERCA2a. Enhanced SERCA2a degradation was not due to alterations in calpain and calpastatin levels or calpain activity. Conversely, HAX-1 overexpression improved contractile recovery and maintained SERCA2a levels. The regulatory effects of HAX-1 on SERCA2a degradation were observed at multiple levels, including intact hearts, isolated cardiomyocytes and sarcoplasmic reticulum microsomes. Mechanistically, HAX-1 ablation elicited increased production of reactive oxygen species at the sarco/endoplasic reticulum compartment, resulting in SERCA2a oxidation and a predisposition to its proteolysis. This effect may be mediated by NAPDH oxidase 4 (NOX4), a novel binding partner of HAX-1. Accordingly, NOX inhibition with apocynin abrogated the effects of HAX-1 ablation in hearts subjected to ischemia/reperfusion injury. Taken together, our findings reveal a role of HAX-1 in the regulation of oxidative stress and SERCA2a degradation, implicating its importance in calcium homeostasis and cell survival pathways.

IL-25 and CD4(+) TH2 cells enhance type 2 innate lymphoid cell-derived IL-13 production, which promotes IgE-mediated experimental food allergy.

BACKGROUND: Food-mediated allergic reactions have emerged as a major health problem. The underlying mechanisms that promote uncontrolled type 2 immune responses to dietary allergens in the gastrointestinal tract remain elusive. OBJECTIVE: We investigated whether altering IL-25 signaling enhances or attenuates allergic responses to food allergens. METHODS: Mice of an IL-25 transgenic mouse line (iIL-25Tg mice), which constitutively overexpress intestinal IL-25, and Il17rb(-/-) mice, in which Il17rb gene expression is disrupted, were sensitized and gavage fed with ovalbumin (OVA). We assessed symptomatic characteristics of experimental food allergy, including incidence of diarrhea, incidence of hypothermia, intestinal TH2 immune response, and serum OVA-specific IgE and mast cell protease 1 production. RESULTS: Rapid induction of Il25 expression in the intestinal epithelium preceded onset of the anaphylactic response to ingested OVA antigen. iIL-25Tg mice were more prone and Il17rb(-/-) mice were more resistant to experimental food allergy. Resident intestinal type 2 innate lymphoid cells (ILC2s) were identified as the major producers of IL-5 and IL-13 in response to IL-25. Reconstituting irradiated wild-type mice with Rora(-/-) or Il17rb(-/-) bone marrow resulted in a deficiency or dysfunction of the ILC2 compartment, respectively, and resistance to experimental food allergy. Repeated intragastric antigen challenge induced a significant increase in numbers of CD4(+) TH2 cells, which enhance IL-25-stimulated IL-13 production by ILC2s ex vivo and in vivo. Finally, reconstituted IL-13-deficient ILC2s had reduced capability to promote allergic inflammation, resulting in increased resistance to experimental food allergy. CONCLUSION: IL-25 and CD4(+) TH2 cells induced by ingested antigens enhance ILC2-derived IL-13 production, thereby promoting IgE-mediated experimental food allergy.

Intraoperative Stereotactic Arteriography in Complex Cervical Spine Surgery.

Intra-operative navigation has revolutionized spinal instrumentation. The O-arm (a mobile X-ray system; Medtronic, Minneapolis, MN) is uniquely capable of enabling visualization of the spine in axial planes. The application of this technology is wide yet underutilized in terms of its capacity to image spinal vascular anatomy. We completed a retrospective chart review of the following case studies. A 24-year-old neurologically intact female presented with a Jefferson fracture without vertebral artery dissection after a motor vehicle accident. After the failure of conservative management due to pseudoarthrosis, the patient opted for fusion. Prior to the procedure, bilateral 5 French femoral sheaths were placed. After exposure, intraarterial (IA) contrast was injected prior to the O-arm spin to visualize both vertebral arteries, which were stretched and adjacent to a mobile boney segment. In the second case, a 71-year-old male presented with right shoulder pain and a flaccid left deltoid secondary to a large enhancing epidural lesion spanning C4-C7. Further work-up confirmed a diagnosis of metastatic intrahepatic cholangiocarcinoma. Prior to resection with cervical spinal stabilization, a right radial artery 4 French Glidesheath was placed. Prior to the O-arm spin, the right vertebral artery was selected, and intravenous contrast was injected to permit visualization of the vertebral artery, which was encased within the tumor and at significant risk for iatrogenic injury. Both patients tolerated the endovascular and spinal procedures well without vertebral artery injury. This is the first series to report the effective use of the O-arm for improved visualization of vascular anatomy during surgery for cervical spinal trauma and oncology.

Multiple three-column osteotomies successfully correcting cervicothoracic kyphosis in the setting of ankylosing spondylitis: illustrative case.

BACKGROUND: Ankylosing spondylitis (AS) is an autoimmune spondylarthritis often associated with rigid kyphoscoliosis. The authors describe a surgical approach that employs multilevel three-column osteotomies for the restoration of normal global alignment. OBSERVATIONS: A 48-year-old male with a past medical history of AS presented to the clinic with a stooped-over posture: his chin-brow vertical angle (CBVA) was 58.0°; T1 slope (T1S), 97.8°; thoracic kyphosis (TK; T1-12), 94.2°; proximal TK (T1-5), 50.8°; distal TK (T5-12), 43.5°; and sagittal vertical axis (SVA), 22.6 cm. A two-stage procedure was planned. During stage 1, instrumentation was placed from C5 to T10, followed by a T3 vertebral column resection. During stage 2, bilateral pedicle screws were placed from T11 to the pelvis. An L3 pedicle subtraction osteotomy (PSO) was completed and was followed by a T7 PSO. Postoperatively, the patient had significant postural improvement: CBVA was 29.3°; T1S, 57.8°; TK, 77.3°; proximal TK, 33.5°; distal TK, 43.8°; and SVA, 15 cm. At 6 years postoperatively, the patient continued to do well and was without evidence of construct breakdown. LESSONS: The authors propose that multilevel three-column osteotomies, if optimally located, successfully correct spinal malalignment associated with AS.

Spinal Meningiomas: A Comprehensive Review and Update on Advancements in Molecular Characterization, Diagnostics, Surgical Approach and Technology, and Alternative Therapies.

Spinal meningiomas are the most common intradural, extramedullary tumor in adults, yet the least common entity when accounting for all meningiomas spanning the neuraxis. While traditionally considered a benign recapitulation of their intracranial counterpart, a paucity of knowledge exists regarding the differences between meningiomas arising from these two anatomic compartments in terms of histopathologic subtypes, molecular tumor biology, surgical principles, long-term functional outcomes, and recurrence rates. To date, advancements at the bench have largely been made for intracranial meningiomas, including the discovery of novel gene targets, DNA methylation profiles, integrated diagnoses, and alternative systemic therapies, with few exceptions reserved for spinal pathology. Likewise, evolving clinical research offers significant updates to our understanding of guiding surgical principles, intraoperative technology, and perioperative patient management for intracranial meningiomas. Nonetheless, spinal meningiomas are predominantly relegated to studies considering non-specific intradural extramedullary spinal tumors of all histopathologic types. The aim of this review is to comprehensively report updates in both basic science and clinical research regarding intraspinal meningiomas and to provide illustrative case examples thereof, thereby lending a better understanding of this heterogenous class of central nervous system tumors.

Severe kyphoscoliosis correction utilizing sublaminar bands in the setting of a vertebral column resection: a unique technical report.

PURPOSE: Severe kyphoscoliosis produces combined coronal and sagittal imbalance, a challenging presentation of Adult Spinal Deformity (ASD). Vertebral column resection (VCR) provides three-dimensional correction typically reserved for severe and/or fixed deformities. The challenge of a VCR is to stabilize the spinal column during incremental reduction maneuvers to correct the deformity. We describe novel use of sub-laminar bands with a temporary pivoting rod construct to achieve coronal and sagittal correction in one reductive maneuver. METHODS: All available notes, imaging, and reports were summarized for inclusion. RESULTS: A 67-year-old male presented severe, progressive thoracolumbar kyphoscoliosis, subsequent back pain, and difficulty in ambulating. Given the progression, surgical intervention was undertaken. The patient underwent an L3 VCR followed by instrumentation from T2 to pelvis. Kyphoscoliosis correction was performed via a single reduction maneuver using novel pivoting-rod construct and sub-laminar bands followed by quad-rod construct placement. Fresh, frozen, femoral head allograft, and BMP were utilized. The patient was placed in a Jewett brace for 6 weeks postoperatively and recovered. His condition remains optimized at three-year follow-up. CONCLUSION: Correcting three-dimensional deformities can require vertebral column resection and pose risk during correction. We propose a sub-laminar band with a pivot-rod construct as a safe, effective technique, limiting the number of reduction maneuvers.

Systematic Review of WHO Grade 4 Astrocytoma in the Cerebellopontine Angle: The Impact of Anatomic Corridor on Treatment Options and Outcomes.

Background Despite advances in multimodal oncologic therapies and molecular genetics, overall survival (OS) in patients with high-grade astrocytomas remains poor. We present an illustrative case and systematic review of rare, predominantly extra-axial World Health Organization (WHO) grade 4 astrocytomas located within the cerebellopontine angle (CPA) and explore the impact of anatomic location on diagnosis, management, and outcomes. Methods A systematic review of adult patients with predominantly extra-axial WHO grade 4 CPA astrocytomas was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines through December 2022. Results Eighteen articles were included comprising 21 astrocytomas: 13 exophytic tumors arising from the cerebellopontine parenchyma and 8 tumors originating from a cranial nerve root entry zone. The median OS was 15 months with one-third of cases demonstrating delayed diagnosis. Gross total resection, molecular genetic profiling, and use of ancillary treatment were low. We report the only patient with an integrated isocitrate dehydrogenase 1 (IDH-1) mutant diagnosis, who, after subtotal resection and chemoradiation, remains alive at 40 months without progression. Conclusion The deep conical-shaped corridor and abundance of eloquent tissue of the CPA significantly limits both surgical resection and utility of device-based therapies in this region. Prompt diagnosis, molecular characterization, and systemic therapeutic advances serve as the predominant means to optimize survival for patients with rare skull base astrocytomas.

Advantages of Short-Segment Fusion in the Surgical Management of Thoracolumbar Traumatic Fractures: A Case Series and Review of the Literature.

INTRODUCTION: Spine trauma is a common pathology that frequently requires neurosurgical intervention. Few studies have examined short-segment, 360-degree stabilization of traumatic thoracolumbar fractures. METHODS: A retrospective review was completed of adult and pediatric patients who underwent surgical correction for thoracolumbar fractures between December 2011 and December 2021. RESULTS: Forty patients met the inclusion criteria. The majority of patients presented with an American Spinal Injury Association (ASIA) score of D (n = 11) or E (n = 21). The most common level of injury was L1 (n = 20). The average length of stay was 11.7 days. Postoperatively, two patients had pulmonary emboli or deep venous thrombosis, and two had surgical site infections. Most patients were discharged to home (n = 21) or acute rehab (n = 14). The fusion rate at six months was 97.5%. Neurologically, all patients regained ambulation by >18 months follow-up. For the ASIA scale, most had a score of D (n = 4) or E (n = 32) at six months. The same trend was observed with the Frankel score, where most patients had either D (n = 5) or E (n = 31), improving to only two having a score of D at >18 months. CONCLUSIONS: Corpectomy followed by posterior fusion has a number of biomechanical benefits. This construct permits circumferential decompression, larger surface area for fusion, improved reconstitution of vertebral body height, reduced kyphosis, and an overall shorter segment. This results in fewer levels needing to be fused while enabling the greatest changes of successful fusion.

Acute Spinal Cord Injury Due to Epidural Lipomatosis Without Osseous Injury.

Spinal epidural lipomatosis (SEL) is a common pathology of the lumbar spine. While the natural history is not well understood, there is a strong association with metabolic syndrome and endocrine dysfunction. Clinical presentation typically involves slow, progressive onset of radicular and myelopathic symptoms. Treatment primarily consists of weight loss, while surgery is reserved for refractory cases or acute cauda equina syndrome. We present a case of acute spinal cord injury (SCI) after trauma with underlying SEL in the cervicothoracic spine. Additionally, a literature review using a MEDLINE search of the English literature through April 2020 following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines was performed to identify all documented cases of acute spinal cord injury with underlying SEL. A 72-year-old obese male with insulin-dependent diabetes mellitus presented with subacute bilateral lower extremity weakness after a fall with a flank injury three days prior to evaluation. Within hours of admission, the patient acutely progressed to paraplegia and sensory loss below the T6 level consistent with an ASIA (American Spinal Injury Association) A spinal cord injury. No fracture or dislocation was identified on CT imaging. MRI of the thoracic spine revealed spinal cord compression secondary to extensive posterior epidural lipomatosis with resultant anterior displacement of the thecal sac. The patient underwent emergent T2-T9 laminectomy for decompression. Post-operatively, the patient regained sensation below the level of injury. A review of the literature reviewed no published articles on cases of complete spinal cord injury secondary to underlying SEL without associated fracture. Finally, we present the first report of an acute spinal cord injury in the setting of SEL without fracture. Our case demonstrates that SEL outside the lumbar spine confers increased risk for SCI following trauma. Patients with cervicothoracic SEL may require close neurological observation and timely surgical decompression.

Intradural Extramedullary Hemangioblastoma of the Cervical Spine: Case Report and Literature Review.

Hemangioblastomas are uncommon in the spine, accounting for less than 3% of all spinal cord tumors and occurring even more rarely in the intradural extramedullary vicinity. We present a unique case report of an intradural extramedullary hemangioblastoma. A 62-year-old man presented with a five-month history of neck pain radiating to the left arm. A magnetic resonance imaging (MRI) of the cervical spine revealed a left paracentral contrast-enhancing intradural extramedullary lesion at the C4-C5 level. Surgical options were discussed, and surgery was performed via a posterolateral approach. The lateral masses and facets at the C4 and C5 levels were drilled and the tumor was encountered ventral to the spinal cord. There were multiple nerve roots adherent to the tumor capsule. The tumor was highly vascularized. Analysis revealed a highly vascular lesion with vacuolated tumor cells, positive for inhibin and S100 stains, consistent with a diagnosis of hemangioblastoma. The patient remains intact throughout the post-operative period. Few studies have reported intradural extramedullary spinal hemangioblastomas and purely extramedullary spinal hemangioblastomas of the neuraxis are far less common. Most cases occur in the Japanese population and in patients over the age of 50. By location, extramedullary hemangioblastomas involving the thoracic spine occur in women, while those occurring in men are restricted to the cervical spine or conus medullaris. Complete resection remains the treatment of choice.

Superior Ophthalmic Vein Flow Patterns as a Marker of Venous Sinus Stenosis and Hypertension in Idiopathic Intracranial Hypertension: A Case of Emergent Transverse Sinus Stenting as Treatment of Fulminant Idiopathic Intracranial Hypertension.

BACKGROUND: Fulminant idiopathic intracranial hypertension (IIH) can cause rapid vision loss. Transverse sinus stenosis is a finding commonly associated with IIH, and transverse sinus stenting has been used to rapidly reduce intracranial pressure and improve visual symptoms. Our objective was to describe a case of immediate alteration in reversed superior ophthalmic vein (SOV) flow in a fulminant IIH patient who underwent venous sinus stenting. METHODS: All charts, imaging, and notes spanning from the initial presentation to the post-intervention follow-up were reviewed and summarized for inclusion. RESULTS: A 24-year-old woman presented with several weeks of severe headaches and progressive vision loss. She was found to have severe papilledema and the opening pressure on lumbar puncture (LP) was 70 mm Hg. Computed tomography (CT) and magnetic resonance imaging demonstrated findings consistent with elevated intracranial pressure, and CT venography revealed stenosis of the right transverse sinus. She underwent an uncomplicated diagnostic cerebral angiogram, right venous sinus manometry, and right transverse to sigmoid sinus stenting procedure. Prior to deployment of the stent, a trans-stenotic pressure gradient of 12 mm Hg was observed within the right transverse-sigmoid junction, and flow through the bilateral SOVs was retrograde. Following stent placement, the pressure gradient normalized, and SOV flow was bidirectional. She subjectively reported improved vision, and there was improving papilledema. Repeat LP yielded an opening pressure of 21.6 mm Hg. CONCLUSIONS: This case demonstrates reversed SOV flow should be considered an indicator of severe venous sinus stenosis, and restoration to normal or near normal state following stenting for IIH is likely indicative of procedural success.

Headache and Other Symptoms in Chiari Malformation Type I Are Associated with Cerebrospinal Fluid Flow Improvement After Decompression: A Two-Institutional Study.

OBJECTIVE: Chiari malformation type I (CMI) is a common neurologic condition and surgery is the standard treatment. We aim to establish the cerebrospinal fluid (CSF) flow change as a potential objective indicator of surgical efficacy to improve symptoms. METHODS: We performed a retrospective 2-center analysis of surgically treated patients with CMI. Qualitative CSF flow studies obtained preoperatively and postoperatively were analyzed by the neuroradiologist, seeking improvement in CSF flow. Symptom status, including headache and neck pain, were recorded. RESULTS: Forty-nine patients were identified between 2010 and 2017. The average age was 36 years, with 47 females. After decompression, CSF flow was improved in 41 patients (group A) and unchanged in 8 (group B). Group A and B had a mean age of 34 and 42 years (P < 0.05) and an average tonsillar herniation of 8.3 and 8.5 mm (P = 0.40), respectively. Group A had improved clinical outcomes, compared with group B (P = 0.024), more specifically, in headache (50% vs. 37%), neck pain (66% vs. 33%), dizziness (78% vs. 50%), vision symptoms (84% vs. 80%), and weakness (100% vs. 66%), respectively. Group B had the only patient who did worse on clinical follow-up. CONCLUSIONS: Patients with CMI often present with a constellation of symptoms. We showed a significant association between improved CSF flow after decompression and symptom alleviation. Further, our study suggests that the presence of improved CSF flow postoperatively could represent an objective indicator for improved patient outcomes.

Osteoporosis as a Risk Factor for Intraoperative Complications and Long-term Instrumentation Failure in Patients With Scoliotic Spinal Deformity.

STUDY DESIGN: A retrospective review study. OBJECTIVE: This study aims to determine the effect of osteoporosis on spine instrumentation. SUMMARY OF BACKGROUND DATA: Osteoporosis is a common skeletal pathology that affects systemic cortical bone maintenance and remodeling. This disease accelerates the degeneration of the spine, often necessitating spinal surgery for progressive vertebral deformity, pathologic fracture, bony canal stenosis, and/or neural element decompression. There is a paucity of literature describing the role of osteoporosis as it relates to both perioperative complications and outcomes after spine fusion surgery. MATERIALS AND METHODS: A retrospective review was conducted of a prospectively maintained database for patients undergoing spine surgery between January 1, 2006 and October 3, 2017. Inclusion criteria included age 18 years and above and surgery performed for the correction of thoracolumbar scoliosis. Data collected included various demographic, clinical, and operative variables. RESULTS: A total of 532 patients met inclusion criteria, including 144 (27%) patients with a diagnosis of osteoporosis. Osteoporosis was significantly associated with increased blood volume loss (P=0.003). Postoperatively, osteoporosis was associated with increased rates of instrumentation failure (19% vs. 10%; P=0.008) and the need for revision surgery (33% vs. 16%; P<0.001). Multivariate analysis confirmed osteoporosis to be an independent risk factor for increased mean number of spinal segments fused (P<0.05), mean blood volume loss (P<0.05), rate of postoperative deep venous thrombosis/pulmonary embolism (P<0.05), rate of instrumentation failure (P<0.05), and need for revision surgery (P<0.05). CONCLUSION: Osteoporosis is a significant risk factor for instrumentation failure and need for revision surgery following arthrodesis for scoliosis correction. Furthermore, patients with osteoporosis have a significantly higher risk of intraoperative blood volume loss and postoperative thromboembolic events.

Lateral Femoral Cutaneous Nerve Angiomyoma.

Angiomyomas are benign tumors derived from smooth muscle cells of vessels. They are principally a pathology of the peripheral nervous system where they involve cutaneous nerves, causing pain and paresthesia. We present a case of a lateral femoral cutaneous nerve angiomyoma and its surgical treatment. A 24-year-old female presented to clinic with right thigh pain in the distribution of the lateral femoral cutaneous nerve, which had been ongoing and progressive for seven months. There was associated numbness and tingling. A lesion was noted in the anterior subcutaneous tissues of the thigh on contrasted CT and MRI. The patient was taken to the operating room where a pearly white lesion was found within the subcutaneous tissue. No effect was seen with stimulation of the lesion. The lesion was removed en bloc, and pathological analysis revealed an angiomyoma. Post-operatively, the patient reported complete resolution of all symptoms, namely, pain and paresthesia. No similar reports were identified within the literature. Together, angiomyomas have been described within the lower extremities to cause pain and paresthesia. This is the first reported case of an angiomyoma involvement within the lateral femoral cutaneous nerve. Complete surgical resection, in this case, was curative and diagnostic.