Malignant Spinal Tumors.

Malignant spinal tumors constitute around 22% of all primary spinal tumors. The most common location of metastases to the spinal region is the extradural compartment. The molecular and genetic characterization of these tumors was the basis for the updated WHO classification of CNS tumors in 2016, where many CNS tumors are now diagnosed according to their genetic profile rather than relying solely on the histopathological appearance. Magnetic resonance imaging (MRI) is the current gold standard for the initial evaluation and subsequent follow-up on intradural spinal cord tumors, and the imaging sequences must include T2-weighted images (WI), short time inversion recovery (STIR), and pre- and post-contrast T1-WI in the axial, sagittal, and coronal planes. The clinical presentation is highly variable and depends on the tumor size, growth rate, type, infiltrative, necrotic and hemorrhagic potential as well as the exact location within the spinal compartment. Surgical intervention remains the mainstay of management of symptomatic and radiographically enlarging spinal tumors, where the goal is to achieve maximal safe resection. Tumor recurrences are managed with repeat surgical resection (preferred whenever possible and safe), radiotherapy, chemotherapy, or any combination of these therapies.

Benign Spinal Tumors.

Benign spinal intradural tumors are relatively rare and include intramedullary tumors with a favorable histology such as low-grade astrocytomas and ependymomas, as well as intradural extramedullary tumors such as meningiomas and schwannomas. The effect on the neural tissue is usually a combination of mass effect and neuronal involvement in cases of infiltrative tumors. The new understanding of molecular profiling of different tumors allowed us to better define central nervous system tumors and tailor treatment accordingly. The mainstay of management of many intradural spinal tumors is maximal safe surgical resection. This goal is more achievable with intradural extramedullary tumors; yet, with a meticulous surgical approach, many of the intramedullary tumors are amenable for safe gross-total or near-total resection. The nature of these tumors is benign; hence, a different way to measure outcome success is pursued and usually depends on functional rather than oncological or survival outcomes.

Transpalpebral transorbital neuroendoscopic (TONES) repair of orbital meningoencephalocele: a technical note.

PURPOSE: Intraorbital encephalocele (OMEC) is a rare entity in adults, usually secondary to an orbital pathology or prior trauma, in particular orbital roof fractures. Treatment of the OMEC is warranted to alleviate the pulsating exophthalmos and prevent potential visual decline. OMEC and orbital roof fractures have been predominantly treated via a craniotomy with a reconstruction of the orbital roof using various implants. With the advances in the endoscopic techniques, neuroendoscopy found its application in the treatment of orbital pathologies. We report a minimally invasive alternative: endoscopic transorbital repair of OMEC. MATERIAL AND METHODS: The repair technique is described with illustrations and clinical images. Narrated operative video demonstrating the procedure is provided. RESULTS: Illustrative case: 50-year-old female presented with progressive right eye proptosis over 6 months. Computed tomography (CT) demonstrated bony erosion in the lateral orbital roof, and magnetic resonance imaging (MRI) showed a small hyperintense T2-weighted and T1-weighted contrast enhancing lesion in the orbit, in the area of the bony erosion. Intraoperatively, the lesion was found to be an orbital encephalocele. The orbital defect was successfully repaired by employing the 'sandwich' technique, in which a dural substitute reinforced with tissue glue were deployed without repair of the osseous orbital roof. The patient tolerated the procedure well with ultimate resolution of proptosis. The cosmetic outcome was excellent. CONCLUSION: The transorbital neuroendoscopic approach (TONES) presents a feasible, minimally invasive alternative treatment option for circumscribed intraorbital encephaloceles with minimal side effects, well tolerated by patients.

Complex craniosynostosis in the context of Carpenter's syndrome.

Carpenter's syndrome or acrocephalopolysyndactyly type II is a rare genetic autosomal recessive disease, with an incidence estimated at 1 per 1 million births. Common findings of a brachydactyly, polysyndactyly, and a trefoil-like skull with extreme brachycephaly due to fusion of the bilateral coronal, sagittal and lambdoid sutures. We report a 12-month-old male who was referred to our care for evaluation of a craniofacial deformity-a trefoil-like skull, flattened and receding forehead, bulging of temporal bones, hypertelorism, exorbitism, and polysyndactyly in the upper and lower limbs and psychomotor delay. Head computed tomography (CT) with 3D reconstruction revealed craniosynostosis with fusion of the coronal, metopic, and sagittal sutures. Correction of the craniofacial deformity was performed with satisfactory aesthesis of the craniofacial bones at 2 years of follow-up. Early correction of craniofacial deformity in Carpenter's syndrome is usually safe within 6 to 12 months. Venous drainage abnormalities and ectatic emissary veins can lead to significant bleeding and may be detected on MR angiography. Significant skull weakening may lead to bony fragmentation while creating cranial flaps and is best evaluated with 3D CT imaging. Taking these pitfalls into consideration decreases the chances of aborting the surgery and may lead to better overall outcomes.

Postoperative magnetic resonance imaging signal changes in middle cerebral peduncle after vestibular schwannoma surgery.

BACKGROUND AND PURPOSE: Preoperative compression of middle cerebellar peduncle (MCP) is often observed in vestibular schwannomas. Its re-expansion is expected after tumour resection, however, frequently its thickness remains unchanged or undergoes further atrophy. Similarly, increased MCP FLAIR signal is often observed and thought to be associated with intraoperative MCP injury. This study investigates the dynamics of MCP FLAIR signal changes over time and their implications in long-term MCP atrophy. MATERIALS AND METHODS: Retrospective analysis of patients operated between 2011 and 2019 was performed. Measurements of FLAIR signals and MCP thickness were performed preoperatively, postoperatively and at follow-up. RESULTS: 28 patients (15 females, mean age 51.94 years) were included. The mean follow-up was 23.98 months. The mean tumour size was 2.99 cm. The MCP FLAIR signal was elevated preoperatively in 10 (35.7%) patients and further increased postoperatively in 22 (78.6%), followed by its decrease at follow up (7 patients, 25%). An immediate postoperative re-expansion of middle cerebellar peduncle was observed in 24 (85.7%) patients. No association between tumour size and preoperative FLAIR was established, however tumour size was negatively associated with the MCP thickness. A significant negative association between a postoperative FLAIR and follow-up thickness (p < 0.001) was noted, even if controlling for tumour size and both tumour size and preoperative MCP thickness. CONCLUSION: In patients with vestibular schwannomas undergoing surgical resection, the middle cerebellar peduncle FLAIR signal seems to associated with long term thickness of MCP, regardless of its initial size, however does not seem to correlate with the clinical outcome.

Spontaneous regression of multiple intracranial capillary hemangiomas in a newborn-long-term follow-up and literature review.

BACKGROUND: Intracranial capillary hemangiomas (ICHs) have a natural history and behavior that is very different from intracranial cavernous malformations. The literature is not consistent as to the best management strategy for ICHs. CASE DESCRIPTION: Our patient is a 40-day-old male infant who presented with progressive increase in head circumference and multiple cutaneous capillary and ICHs. Obstructive hydrocephalus necessitated urgent cerebrospinal fluid (CSF) diversion, but no other surgical intervention was pursued due to the high risk-to-benefit ratio. All intracranial lesions spontaneously regressed by 11 years of age, albeit at a slower speed than the cutaneous lesions, with no functional or cognitive sequelae. We conducted a comprehensive literature review and provided a summary of all reported ICH cases. CONCLUSION: Asymptomatic patients with ICHs are best approached with close follow-up and serial imaging studies as the potential for spontaneous regression is relatively high. Patients with isolated lesions or unclear diagnoses may benefit from a stereotactic biopsy, and surgical resection should be reserved for symptomatic lesions only.

Percutaneous lumbar pedicle fixation in young children with flexion-distraction injury-case report and operative technique.

Thoracolumbar fractures in children are relatively uncommon and should be regarded as a separate entity from those in adults. While percutaneous pedicle fixation has emerged as an effective alternative to open fixation in adults with unstable thoracolumbar fractures, this technique is rarely applied in children. We report a 6-year-old girl with an L3 chance fracture, which was treated via short-segment percutaneous pedicle fixation. We also discussed the technical challenges and caveats of this surgical technique in young children. While potentially more challenging, percutaneous pedicle fixation is feasible in young children with thoracolumbar fractures. Specific differences between the developing and mature spine in regard to anatomical and biomechanical characteristics, including ligamentous laxity and intrinsic elasticity, should be taken into consideration. Future studies are needed to compare outcomes of minimally invasive spinal techniques to open surgery in children.

A glimpse into the history of description of the antiphospholipid syndrome.

Prior to 1983, several landmark reports prepared the stage for a detailed description of the Antiphospholipid (Hughes) syndrome (APS). Formerly depicted as lupus-like, APS exhibits a wide spectrum of symptoms that overlap with Sjogren's, Hashimoto, and other autoimmune diseases. In this review, we take a glimpse into the history of description of APS, discussing the events that led to its recognition as one of the most common autoimmune diseases and the enormous impact of that recognition in the rheumatology field.

Management of antiphospholipid syndrome.

Antiphospholipid syndrome, also known as 'Hughes Syndrome', is an autoimmune disease characterised by a set of clinical manifestations, almost all of which are direct or indirect sequelae of a hypercoagulable state involving the venous, and to a lesser extent the arterial vasculature. The incidence and prevalence of antiphospholipid syndrome are estimated at approximately 5 de novo cases per 100 000 per year and 40-50 cases per 100 000 individuals, respectively. The clinical spectrum of antiphospholipid syndrome involves haematological (thrombocytopaenia, venous thrombosis), obstetrical (recurrent pregnancy loss), neurological (stroke, transient ischaemic attack, migraine, seizures, cognitive dysfunction, chorea, transverse myelitis, multiple sclerosis), cardiovascular (cardiac valve disease), dermatological (livedo reticularis and racemosa, skin ulceration and necrosis), renal (glomerulonephritis, renal thrombotic microangiopathy) and orthopaedic (avascular necrosis of bones, non-traumatic fractures) manifestations, among others. In addition to the classical antiphospholipid antibodies, namely anticardiolipin antibodies and lupus anticoagulant, new autoantibodies and antibody complexes of different immunoglobulin subtypes (IgA, IgG, IgM) are now recognised as significant contributors to the pathogenesis of antiphospholipid syndrome. Anticoagulation remains the cornerstone in the management of antiphospholipid syndrome; nevertheless, new drugs and therapeutic strategies are being tested, and some have been found effective for the primary and secondary thromboprophylaxis in antiphospholipid syndrome.

Scoliosis in patients with Chiari malformation type I.

The literature about the association between Chiari malformations (CMs) and scoliosis has been growing over the last three decades; yet, no consensus on the optimal management approach in this patient population has been reached. Spinal anomalies such as isolated syrinxes, isolated CM, and CM with a syrinx are relatively common among patients with presumed idiopathic scoliosis (IS), a rule that also applies to scoliosis among CM patients as well. In CM patients, scoliosis presents with atypical features such as early onset, left apical or kyphotic curvature, and neurological deficits. While spinal X-rays are essential to confirm the diagnosis of scoliosis among CM patients, a magnetic resonance imaging (MRI) is also recommended in IS patients with atypical presentations. Hypotheses attempting to explain the occurrence of scoliosis in CM patients include cerebellar tonsillar compression of the cervicomedullary junction and uneven expansion of a syrinx in the horizontal plane of the spinal cord. Early detection of scoliosis on routine spinal examination and close follow-up on curve stability and progression are essential initial steps in the management of scoliosis, especially in patients with CM, who may require full spine MRI to screen for associated neuro-axial anomalies; bracing and spinal fusion may be subsequently pursued in high-risk patients.

Rheumatological complications of beta-thalassaemia: an overview.

Beta-thalassaemia, an autosomal recessive haemoglobinopathy, ranks among the most frequent monogenetic diseases globally. The severe form of the disease, beta-thalassaemia major, is accompanied by progressive involvement of multiple organ systems as a result of the disease pathophysiology as well as iron overload from blood transfusions on a regular basis. Some of the manifestations might also be caused by medications used to manage iron overload. The purpose of this review is to highlight the rheumatological complications of beta-thalassaemia, which include musculoskeletal manifestations, such as arthritis and arthropathies, joint effusions, osteoporosis, bone fractures and myalgias, in addition to CTDs, such as pseudoxanthoma elasticum. Rheumatologists are strongly encouraged to take part in a multidisciplinary approach to the management of this debilitating disease.

Hyperacoustic hypoacusis: A new pontine syndrome-Case report.

We herein report a unique case of a lateral pontine demyelinating lesion presenting with unilateral sensorineural hearing loss and paradoxical ipsilateral hyperacusis. The association of unilateral hearing loss and ipsilateral hyperacusis is a rare manifestation of a central nervous system lesion. The paradoxical combination of these symptoms strongly suggests pontine dysfunction and prompts urgent neurological evaluation.

Antiphospholipid syndrome: an update.

BACKGROUND: Antiphospholipid syndrome (APS) or 'Hughes syndrome' is a prothrombotic disease characterized by thrombosis and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). More than three decades have passed, and experts are still uncovering new pieces of this disease complex pathogenesis and management. MATERIALS AND METHODS: We searched in literature using MEDLINE and PubMed databases focusing on the latest development on disease pathogenesis, risk assessment of thrombosis and treatment of APS. RESULTS: The phosphatidylinositol 3-kinase (PI3K)-AKT-mTORC pathway was most recently identified to have a crucial role in activating inflammation among endothelial vessel wall causing vascular lesions in APS. Additionally, new variables are being implemented to assess the risk of thrombosis in patients with APS. Global APS Score (GAPSS) utilizes cardiovascular risk factors and new autoimmune antibodies as part of the score assessment and is the most valid so far. It can be a promising tool in the future for prediction of thrombosis. Anticoagulation remains the cornerstone in APS; however, many new potential therapeutic agents are developing and are currently under investigation. CONCLUSIONS: The most recent advances in pathogenesis, risk stratification and treatment provide a platform for high yield studies with the ultimate goal of providing the optimal management to patients with APS.

Pelvic Incidence as a Predictor of Proximal Junctional Failure in Patients Undergoing Anterior Column Realignment with Anterior Longitudinal Ligament Release to Restore Lordosis in Adult Spinal Deformity: A Retrospective Cohort Study.

OBJECTIVE: To identify variables that may contribute to the development of proximal junctional failure (PJF) in patients with long lumbo-sacral and thoraco-lumbo-pelvic constructs undergoing anterior column realignment (ACR) with anterior longitudinal ligament release (ALLR). METHODS: Data of patients with adult spinal deformity who underwent ACR with ALLR at L3-4 were collected retrospectively from medical records and a prospectively maintained spine research database between 2016 and 2022. RESULTS: Eleven (41%) developed PJF at a mean of 24 ± 21 months from the index surgery. The cohort was then divided into 2 groups for analysis, 13 subjects in the high pelvic incidence (PI) group (defined as PI ≥ 55°) and 14 subjects in the low PI group (defined as PI < 55°). Visual Analog Scale for back pain and Oswestry Disability Index decreased from 9.5 to 2.1 and 61 to 10 in the high PI group, and from 8.9 to 2.4 and 60.9 to 10.3 in the low PI group, respectively. PI (P = 0.004), sacral slope (P = 0.005), and postoperative PI-lumbar lordosis mismatch (P = 0.02) were found to be significant predictors of PJF. The receiver operator curve revealed a cutoff PI value ≤ 53° (95% confidence interval: 52°-64°), below which the risk of PJF becomes significantly higher in patients undergoing ACR with ALLR at L3-4. CONCLUSIONS: PI may be a predictor of PJF and highly correlates with ACR-ALLR levels. In patients undergoing L3-4 ACR-ALLR, a PI value of ≤53° is associated with a significantly elevated risk of PJF. Preoperative planning of ACR-ALLR level based on normal sagittal alignment in otherwise healthy individuals may mitigate the risk of PJF development in patients with adult spinal deformity treated with ACR-ALLR.

The Role of Oxidative Stress in the Progression of Secondary Brain Injury Following Germinal Matrix Hemorrhage.

Germinal matrix hemorrhage (GMH) can be a fatal condition responsible for the death of 1.7% of all neonates in the USA. The majority of GMH survivors develop long-term sequalae with debilitating comorbidities. Higher grade GMH is associated with higher mortality rates and higher prevalence of comorbidities. The pathophysiology of GMH can be broken down into two main titles: faulty hemodynamic autoregulation and structural weakness at the level of tissues and cells. Prematurity is the most significant risk factor for GMH, and it predisposes to both major pathophysiological mechanisms of the condition. Secondary brain injury is an important determinant of survival and comorbidities following GMH. Mechanisms of brain injury secondary to GMH include apoptosis, necrosis, neuroinflammation, and oxidative stress. This review will have a special focus on the mechanisms of oxidative stress following GMH, including but not limited to inflammation, mitochondrial reactive oxygen species, glutamate toxicity, and hemoglobin metabolic products. In addition, this review will explore treatment options of GMH, especially targeted therapy.

Biochemical Markers of Early Renal Dysfunction in Patients with ß-thalassemia Major: A Systematic Review and Meta-analysis.

BACKGROUND: Early diagnosis of renal dysfunction in ß-thalassemia major (ß- TM) may help take specific measures to delay irreversible damage and renal failure. Therefore, the present meta-analysis aimed to compare biochemical markers of premature renal dysfunction between ß-TM and healthy subjects and identify renal issues' prevalence in patients with ß-TM. METHODS: We searched PubMed, Cumulative Index of Nursing and Allied Health Literature (CINAHL), Medline, Scopus, Web of Science, ScienceDirect, ProQuest, Google Scholar, and State Inpatient Databases (SIDs) without any language constraints for all relevant articles published up to April 2019. RESULTS: Out of 1458 articles published up to April 2019, 24 case-control and 22 crosssectional studies were investigated. The investigated levels of serum phosphorus, uric acid (UA), cystatin C, and ferritin were significantly different between ß-TM patients and controls. The albumin/creatinine ratio (ACR), N-acetyl-ß-D-glucosaminidase/creatinine (NAG/Cr) ratio, urinary and serum ß2 microglobulin (ß2MG), and serum ferritin levels were significantly higher in ß-TM patients than in healthy individuals. However, glomerular filtration rate, creatinine clearance, and pretransfusion hemoglobin indicated a significantly lower rate. The general prevalence of renal glomerular and/or tubular defects in patients with ß-TM was 50.22%. CONCLUSION: Urinary NAG, ß2MG, ACR, and Scys-C may be early markers of renal dysfunction in patients with ß-thalassemia major. An observation of elevated levels of these markers despite normal levels of other markers of renal dysfunction may indicate primary, subclinical injury to the renal tubules and glomeruli.

Improved Outcomes with Concurrent Instrumentation and Fusion of the Sacroiliac Joint in Patients with Long Lumbosacral Constructs.

STUDY DESIGN: Retrospective Cohort Study. OBJECTIVE: Spinal fusion, specifically constructs connected to pelvic bones, has been consistently reported as a predisposing factor to sacroiliac joint (SIJ) pain. The aim of this study is to compare SIJ outcomes in patients with constructs to the pelvis following instrumentation vs instrumentation plus fusion of the SIJ. METHODS: Data of study subjects was extracted from a prospectively maintained database as well as retrospectively collected from records at a tertiary academic medical center in the United States between 2018 and 2020. RESULTS: A cohort of 103 patients was divided into 2 groups: 65 in Group 1 [S2AI screw without fusion device] and 38 in Group 2 [S2AI screw with fusion device]. None of the patients in Group 2 developed postoperative SIJ pain compared to 44.6% in Group 1. Sacroiliac joint fusion occurred in all Group 2 but none of Group 1 patients. The postoperative Visual Analogue Scale (VAS) for lower extremity (LE) pain (.8 vs .5; P = .03) and postoperative Oswestry Disability Index (ODI) (18.7 vs 14.2; P < .01) were significantly higher in Group 1. The rate of distal junctional break, failure, and/or kyphosis (DJBFK) and time to DJBFK were not significantly different between the two groups, and the rate of DJBFK did not change in the presence of multiple covariates. CONCLUSION: The SIJs carry the heavy load of long lumbosacral fusion constructs extending to the pelvis. Simultaneous SIJ instrumentation and fusion decreases the risk of disability, prevents the development of postoperative SIJ pain, and may also protect the S2AI screw from loosening and failure.

Modern Appraisal of Patency and Complications in Cerebral Bypass Surgery: A Single Institution Experience.

BACKGROUND: Cerebral bypass is a valuable surgical technique in well-selected patient populations. Updated clinical guidelines and improved surgical techniques warrant a contemporary reevaluation of the complications and patency to inform clinical practice and enhance postoperative patient care. OBJECTIVE: To assess the complication rates and postoperative graft patency for the 3 most common indications for bypass surgery: moyamoya disease, intracranial atherosclerosis, and intracranial aneurysms. METHODS: Perioperative notes of 175 consecutive bypass patients at a single institution were retrospectively identified to evaluate the clinical course and complications of surgery. RESULTS: The rate of total postoperative complications between moyamoya disease (9 of 98, 9.2%), intracranial atherosclerotic disease (7 of 57, 12.3%), and intracranial aneurysm (4 of 20, 20%) was not statistically different (P = .33). Immediate postoperative bypass patency was significantly higher in moyamoya disease (90 of 96, 93.8%) and intracranial atherosclerotic disease (48 of 51, 94.1%) than in intracranial aneurysm (13 of 18, 72.2%; P = .02). Intravenous heparin administration during bypass suturing was negatively associated with immediate postoperative patency (87% heparin patency vs 99% no heparin patency; P = .02). Double-barrel bypass trended toward an increased risk of wound healing complications (2 of 13, 15.4%) compared with the single-barrel bypass technique (4 of 156, 2.6%; P = .07). CONCLUSION: Cerebral bypass surgery remains an excellent surgical treatment for moyamoya disease, intracranial atherosclerosis, and intracranial aneurysms. This study suggests bypass is safer in moyamoya disease and intracranial atherosclerosis. Additional studies to clarify the risk of single-barrel vs double-barrel bypass and intraoperative heparin-stratified complications may be beneficial.

Neuroendoscopic Transventricular Approach for Cystic Craniopharyngioma.

The literature is rich with many studies reporting different treatment modalities and approaches for cystic craniopharyngioma (CC), including microsurgery, neuroendoscopic transventricular approach, endoscopic transnasal surgery, stereotactic drainage, and Ommaya reservoir insertion. The goals of this manuscript are to report the successful treatment of an atypical case of CC using the neuroendoscopic transventricular approach (NTVA) as well as discuss the different surgical modalities for these tumors following a comprehensive review of the literature. Our patient is a nine-year-old female with a large CC who was managed using the NTVA. No complications or recurrence occurred over two years of follow-up. Results of our literature review showed lower recurrence and complication rates of the NTVA compared to other surgical modalities.The NTVA is potentially efficient, reliable, and safe for managing CC and cystic-dominant craniopharyngiomas, with low recurrence and complication rates compared to microsurgery and Ommaya reservoir insertion. Future randomized clinical studies comparing the various treatment modalities of CC are needed to solidify these conclusions.

Predicting neurosurgical clearance in the polytrauma patient with concomitant traumatic brain injury.

The goal of this study is to develop a model based on previously used prognostic predictors in traumatic brain injury (TBI) patients with polytrauma, which will facilitate the decision-making of whether to clear these patients for non-cranial surgery. Data of eligible patients was obtained from a trauma database at a Level I trauma and academic tertiary referral center in the United States. The number of days seen by the neurosurgical service prior to clearance, injury severity score (ISS), post-trauma day 0 (PTD 0) of Glasgow Coma Score (GCS), intracranial pressure (ICP) score and computed tomography (CT) score, as well as the changes in GCS, ICP score and CT score between PTD 0 and day of clearance were the variables used in developing the model. The Neurosurgical Clearance Model (NCM) was developed using data from 50 patients included in the study. Patients were cleared by neurosurgeons 1.6 days later than it would appear possible based on a retrospective review of the patients' clinical conditions. A single model equation was developed, the ultimate result of which is a clearance probability value. The best cutoff clearance probability value was found to be 0.584 (or 58.4%) using Receiver Operator Characteristic curve analysis. Our data suggests that neurosurgeons are risk-averse in clearing polytrauma patients for non-cranial surgery. This pilot NCM, if reproduced and validated by other groups and in larger prospective studies, may become a useful tool to assist clinicians in this often-difficult decision-making process.