Supraclinoid Internal Carotid Artery Dolichoectasia Causing Compression of the Prechiasmatic Optic Nerve.

BACKGROUND AND IMPORTANCE: Intracranial arterial dolichoectasia (or dilatative arteriopathy) is characterized by abnormal elongation, tortuosity, or increase in diameter of at least one of the main cerebral vessels. Dolichoectasia can be found incidentally or can present with cranial neuropathies (including vision loss) or stroke. Here, we describe the presentation and open surgical treatment of a patient with dolichoectasia of the intracranial internal carotid artery (ICA) causing monocular vision loss. CLINICAL PRESENTATION: A 73-year-old man presented with several months of progressive monocular vision loss and was found to have dolichoectasia of the supraclinoid ICA and subsequently underwent microsurgical decompression of the overlying affected optic nerve. The patient's postoperative convalescence was uncomplicated, and he had improvement in his right-sided monocular vision loss after surgery. CONCLUSION: We present the case of a patient with dolichoectasia of the supraclinoid ICA causing compression of the optic nerve with resultant monocular vision loss. Timely microvascular decompression proves to be an effective technique for vision preservation in the setting of this rare pathologic entity.

Natural history of pituitary carcinoma with metastasis to the cervical spine: illustrative case.

BACKGROUND: Pituitary carcinoma is a rare tumor of the adenohypophysis with noncontiguous craniospinal dissemination and/or systemic metastases. Given the rarity of this malignancy, there is limited knowledge and consensus regarding its natural history, prognosis, and optimal treatment. OBSERVATIONS: The authors present the case of a 46-year-old woman initially treated with invasive prolactin-secreting pituitary macroadenoma who developed metastatic disease of the cervical spine 6 years later. The patient presented with acutely worsening compressive cervical myelopathy and required posterior cervical decompression, tumor resection, and instrumented arthrodesis for posterolateral fusion. LESSONS: This case underscores the importance of long-term monitoring of hormone levels and having a high clinical suspicion for metastatic disease to the spine in patients presenting with acute myelopathy or radiculopathy in the setting of previously treated invasive secreting pituitary adenoma.

High-grade glioma imaging volumes and survival: a single-institution analysis of 101 patients after resection using intraoperative MRI.

PURPOSE: Intraoperative magnetic resonance imaging (iMRI) has been efficacious in maximizing resection of high-grade gliomas (HGGs). In this single-institution study of patients with HGGs who underwent resection using iMRI, the authors present a volumetric-based survival analysis to evaluate progression-free survival (PFS) and overall survival (OS), as well as the impact of additional resection on survival. METHODS: This retrospective analysis included patients with HGGs who underwent resection using iMRI from 2011 to 2021. Volumetric analyses of T1-weighted contrast-enhancing (T1W-CE), T2-weighted (T2W), and T2W fluid-attenuated inversion recovery (FLAIR) MRI sequences were assessed at preoperative, intraoperative, immediate postoperative, and three-month postoperative timepoints. Statistical analyses were carried out using log-rank and multivariable Cox proportional hazard regression analyses. RESULTS: A total of 101 patients (median age 57.0 years) were treated. In keeping with prior studies, statistically significant associations between greater EOR and longer PFS and OS were seen (p = 0.012 and p = 0.006, respectively). The results demonstrated significant associations of lower preoperative T2W, 3-month postoperative T2W, and 3-month postoperative FLAIR volumes with longer PFS and OS (p = 0.045 and p = 0.026, p = 0.031 and p = 0.006, p = 0.018 and p = 0.004, respectively), as well as associations between lower immediate postoperative T2W and immediate postoperative FLAIR volumes with longer OS (p = 0.002 and p = 0.02). There was no observed association in either PFS or OS for patients undergoing additional resection after initial iMRI scan (p = 0.387 and p = 0.592). CONCLUSION: This study of 101 patients with new or recurrent HGGs shows three-month postoperative T2W and FLAIR imaging volumes were significant prognosticators with respect to PFS and OS.

Pediatric Traumatic Brain Injury in a Geographically Dispersed Population: A Relationship Between Distance to Definitive Neurosurgical Treatment and Outcome.

BACKGROUND: There are limited data on the association between transport distance and outcomes in pediatric patients with severe traumatic brain injuries (sTBIs), despite children having to travel further to pediatric trauma centers (PTCs). OBJECTIVE: To assess whether distance from a PTC is associated with outcomes in children who undergo cranial surgery after sTBI. METHODS: Children with sTBI who underwent craniectomy/craniotomy at our PTC between 2010 and 2019 were identified retrospectively. Of these 92 patients, 83 sustained blunt injury and underwent surgery within 24 hours. The distance from injury location to PTC was based on injury zip code and calculated as Euclidean distance. Variables associated with transport, including distance, time, and rural-urban disparity, were analyzed for correlation with poor outcome. RESULTS: Of the 83 patients identified, 81 had injury location information. Forty patients were injured within 30 miles and 41 were injured ≥30 miles from the PTC. Injury severity and pediatric trauma scores were not significantly different between groups. Sixty-eight children (82%) had a satisfactory outcome and 10 children (12%) died. There was a nonsignificant association between distance traveled and poor outcome, even when the cohort was stratified into those with subdural hematomas and those with nonabusive injuries. CONCLUSIONS: Regardless of the distance from the PTC at which their injury occurred, most children in this cohort made a moderate to good recovery. Children injured at greater distances from the PTC did not have worse outcomes; however, studies with larger cohorts are needed to more definitively assess prehospital pediatric transport systems in this population.

Carotid Stump Syndrome With Stent-Assisted Coil Embolization.

Carotid stump syndrome (CSS) is a rare cause of recurrent ipsilateral cerebrovascular events that typically manifests as transient ischemic attacks or amaurosis fugax. The cause of these recurrent symptoms is thought to be microembolization from an occluded internal carotid artery that reaches intracranial circulation through anastomoses. We undertook a systematic literature review according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using the PubMed, Web of Science, and Embase databases of the endovascular treatment options for CSS. Nine papers met the inclusion criteria and provided patient data on 12 patients, and one case illustration is presented. Treatment was with common carotid artery-external carotid artery stent graft without concomitant coil embolization in nine patients and with coil embolization without stenting, the breakthrough of the stump with a wire and subsequent internal carotid artery stent placement, and stent-assisted coil embolization in one patient each. During a median follow-up of six months, all patients were on dual antiplatelet therapy except one on undefined "systemic anticoagulation." Twelve patients had no symptoms after treatment, one had transient expressive aphasia but no further symptoms after being placed on anticoagulation, and none had intraprocedural complications or had to undergo retreatment. Our review indicates that endovascular treatment of CSS is associated with low intraprocedural risk and is effective at treating recurrent symptoms.

Solitary manifestations of primary B-lymphoblastic lymphoma of the spine: Systematic literature review with case illustration.

BACKGROUND: Given the locally destructive osteolytic nature of primary B-cell lymphoblastic lymphoma (B-LBL) of the spine, careful attention is needed to identify clinical signs and symptoms as well as radiological findings of spinal instability because these lesions may warrant resection, decompression, and instrumentation with posterolateral fusion. Our objective was to summarize the presenting symptoms, clinical features, potential treatment modalities, and clinical outcome of cases described in the literature. METHODS: We undertook a systematic literature review to identify all relevant cases and case series describing primary manifestations B-LBL of the spine using Pubmed/Medline. We summarized the findings in accordance with the PRISMA guidelines. We also present a case illustration. RESULTS: Together with our case, 9 cases of primary B-LBL of the spine were identified in 6 male and 3 female patients (age 8-58 years, median 31 years). Back pain was the most common symptom, and five patients also had neurological signs of spinal cord compression. T1-weighted MRI contrast enhancement was seen in 5 cases. Surgery was performed in 5 patients with progression of neurological deficits. Steroid treatment was also given in 3 patients preoperatively. Seven patients had chemotherapy after diagnosis. During follow-up of 1 month to 1 year, 2 cases of recurrence and 4 cases of complete remission were noted; however, with the short follow-up time, patient prognosis overall remains unclear. CONCLUSIONS: Primary B-LBL of the spine represents a rare clinical entity whose management mandates a multidisciplinary approach. Careful attention must be paid to the neurological status of the patient, as well as to imaging that may highlight potential local instability of the spine.

Impact of modified Frailty Index-11 (mFI-11) on postoperative complications in patients undergoing transsphenoidal resection of pituitary tumors: Analysis of 2006-2014 ACS-NSQIP database.

INTRODUCTION: Frailty is a measure of physiologic reserve that is frequently cited as a predictor of postoperative complications. However, the effect of frailty on patients undergoing a relatively common procedure such as transsphenoidal resection of pituitary tumors (TSRPT) is unknown. Therefore, we sought to explore this relationship using a large, national database. METHODS: The 2006-2014 American College of Surgeons National Surgical Quality Improvement Program database was retrospectively reviewed to identify all patients who underwent TSRPT. Frailty scores were assigned using the established 11-factor modified Frailty Index (mFI-11). Patients were divided into low-frailty and high-frailty groups, based on mFI comorbidities of ≤ 1 and ≥ 2, respectively. Univariable and multivariable analyses were performed to evaluate the impact of frailty on postoperative outcomes and mortality. RESULTS: A total of 993 patients were included in the analysis. The low-frailty group consisted of 825 patients; the high-frailty group comprised 168 patients. In univariable analysis, there were no significant differences in medical (low-frailty 4.8%, high-frailty 8.3%; p = 0.069) and surgical (low-frailty 1.1%, high-frailty 1.2%; p = 1.000) complications; however, the high-frailty group had a higher rate of mortality (3%) when compared with the low-frailty group (0.6%; p = 0.016, OR 4.07, p = 0.044) and longer hospitalization (4.5 ±â€¯7.4 vs. 5.8 ±â€¯6.8 days; p = 0.023). In multivariable analysis, frailty was a predictor of mortality but not complications or reoperation. CONCLUSIONS: Our study shows that frailty, as measured by the mFI-11, does not predict postoperative complications in patients who undergo TSRPT, but greater frailty is correlated with higher mortality and increased hospital length of stay.

Stereotactic laser interstitial thermal therapy for brainstem cavernous malformations: two preliminary cases.

Brainstem cavernous malformations (CMs) often have high hemorrhage rates and significant posthemorrhage morbidity. The authors present two cases in which magnetic resonance thermography-guided laser interstitial therapy was used for treatment of pontine CMs after recurrent hemorrhage. Both patients showed significant symptomatic improvement and were hemorrhage-free at 12- and 6-month follow-up, respectively. Each had radiographic evidence of lesion involution on serial follow-up imaging. These early results demonstrate this treatment modality may be technically safe; however, larger case numbers and longer follow-up are needed to demonstrate efficacy.

Effect of patient age on glioblastoma perioperative treatment costs: a value driven outcome database analysis.

INTRODUCTION: Identification of groups of patients or interventions with higher associated treatment costs may be beneficial in efforts to decrease the overall financial burden of glioblastoma (GBM) treatment. The authors' objective was to evaluate perioperative surgical treatment cost differences between elderly and nonelderly patients with GBM using the Value Driven Outcome (VDO) database. METHODS: The authors obtained data from a retrospective cohort of GBM patients treated surgically (resection or biopsy) at their institution from August 2011 to February 2018. Data were compiled using medical records and the VDO database. RESULTS: A total of 181 patients with GBM were included. Patients were grouped into age < 70 years at time of surgery (nonelderly; n = 121) and ≥ 70 years (elderly; n = 60). Costs were approximately 38% higher in the elderly group on average (each patient was mean 0.68% of total cohort cost vs. 0.49%, p = 0.044). Higher age significantly, but weakly, correlated with higher treatment cost on linear regression analysis (p = 0.007; R2 = 0.04). Length of stay was significantly associated with increased cost on linear regression (p < 0.001, R2 = 0.84) and was significantly longer in the elderly group (8.7 ± 11.3 vs. 5.2 ± 4.3 days, p = 0.025). The cost breakdown by facility, pharmacy, supply/implants, imaging, and laboratory costs was not significantly different between age groups. Elderly patients with any postoperative complication had 2.1 times greater total costs than those without complication (p = 0.094), 2.9 times greater total costs than nonelderly patients with complication (p = 0.013), and 2.3 times greater total costs than nonelderly patients without complication (p = 0.022). CONCLUSIONS: GBM surgical treatment costs are higher in older patients, particularly those who experience postoperative complications.

Preoperative and intraoperative perfusion magnetic resonance imaging in a RELA fusion-positive anaplastic ependymoma: A case report.

BACKGROUND: Ependymomas are rare neuroepithelial tumors thought to arise from radial glial precursor cells lining the walls of the ventricles and central canal of the brain and spinal cord, respectively. Histopathological classification, according to World Health Organization criteria, has only recently defined the RELA-fusion positive ependymoma. These tumors may account for 70% of supratentorial ependymomas in children and represent an aggressive entity distinct from other ependymomas. CASE DESCRIPTION: Here we present the case of a patient with RELA-fusion positive ependymoma of the frontal lobe in whom we used preoperative and intraoperative magnetic resonance (MR) perfusion imaging. In this first demonstrated intraoperative evaluation of MR perfusion in ependymoma, increased peripheral perfusion of the lesion in a ring-like manner with a discrete cutoff around the surgical margin correlated with intraoperative findings of a clear border between the tumor and brain, as well as pathological findings of increased MIB index and hypercellularity-specifically within solid tumor components. An abnormal perfusion pattern also suggested an aggressive lesion, which was later confirmed on pathological analysis. In addition, intraoperative MR perfusion improved detection of tumor tissue in combination with traditional T1-weighted contrast-enhanced methods, which increased extent of resection. CONCLUSIONS: MR perfusion imaging may be a useful method for delineating tumor aggressiveness and borders, which can be prognostic.

Giant Cell Tumor of the Lateral Skull Base: Diagnostic and Management Options.

Giant cell tumor of bone (GCTB) is a rare, benign, osteolytic neoplasm that most commonly occurs in early adulthood and often involves the long bones of the body. Although GCTB largely affects the epiphyses of long bones, several reports of GCTB involvement of the cranial and facial bones exist in the literature. In addition to reviewing other reported cases of GCTBs of the lateral skull base in the literature, the authors report here on the clinical presentation, radiographic findings, and neurosurgical management of a patient found to have a GCTB of the middle and infratemporal fossae, which was treated by aggressive en bloc resection of the lateral skull base.

Clinical presentation and treatment paradigms in patients with hereditary hemorrhagic telangiectasia and spinal vascular malformations.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder that causes angiodysplasia and results in mucocutaneous telangiectasias and arteriovenous malformations of organs. Although central nervous system vascular malformations can occur anywhere along the neuraxis, spinal vascular malformations are rare. We present our experience with the presentation and management of spinal vascular malformations in patients with HHT. Of the more than 800 patients with the diagnosis of HHT screened at our institution from 1995 through 2017, four patients with spinal vascular malformations (age range 1 month-77 years; 2 male, 2 female) were identified, three of whom came to clinical attention after significant neurological deterioration from previously unknown malformations. A review of the literature including our patients demonstrated 29 total spinal arteriovenous fistulas (AVFs) in 28 HHT patients (69% male). The lesions were located predominantly in the thoracic spine (65.5%). Three lesions were not treated, 17 were treated with embolization, 6 were surgically resected, and 3 were treated with embolization and surgery. In 14 cases, the patients presented with hemorrhage of the AVF. Overall, 79% of patients achieved complete or near-complete occlusion, with 75% reporting improvement in neurological function. Discovery of spinal lesions often occurs after neurological decline because current screening protocols do not include evaluation of the patient for spinal lesions. Most patients benefit from intervention, which is tailored to the characteristics of the patient and their malformation. Given the often-severe neurological deficit encountered at presentation, we favor a protocol that screens HHT patients for spinal vascular malformations.

Acute Compartment Syndrome as a Complication of the Use of Intraoperative Neuromonitoring Needle Electrodes.

BACKGROUND: The use of intraoperative neurophysiologic monitoring (IOM) has become commonplace in many neurosurgical procedures as a tool to reduce the risk of complications through the early identification of reversible neurologic compromise. Although complications related to IOM itself are exceedingly rare, recognizing their clinical presentation in the postoperative neurosurgical patient is essential for the early identification and implementation of appropriate treatment. CASE DESCRIPTION: The authors present a case report of a patient who developed postoperative acute compartment syndrome in the right arm after placement of neuromonitoring needles for routine IOM during endovascular treatment of a left internal carotid artery aneurysm. Before the procedure, the patient received dual antiplatelet therapy and was noted to have a P2Y12 reaction unit value within therapeutic range. The patient had not received other anticoagulation therapy and had no family or personal history of hematologic or coagulopathic disorders. Immediately after an uncomplicated endovascular intervention, the patient began to develop symptoms of forearm swelling, tightness, and tenderness to palpation; pain with wrist flexion and extension; and paresthesias of the distal digits of the hand. She had eventual loss of a palpable radial pulse. The patient underwent emergent fasciotomies of multiple forearm compartments and had immediate return of a palpable radial pulse. CONCLUSIONS: This case represents the first report of post-procedural compartment syndrome resulting from placement of neuromonitoring needles for routine IOM. Although it is a particularly rare complication of IOM, compartment syndrome represents a surgical emergency that carries significant morbidity if not immediately recognized and treated.

Clinical presentation and treatment paradigms of brain arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT) is characterized by recurrent spontaneous epistaxis, mucocutaneous telangiectases, and multisystem arteriovenous malformations (AVMs). Brain AVMs typically present at birth and are identified in approximately 10-20% of patients with HHT. A retrospective review was undertaken of all HHT patients with known single or multiple brain AVMs treated at our institution. Thirty-nine patients with brain AVM(s) were diagnosed with HHT. Most patients presented with at least one Curaçao criterion. A total of 78 brain AVMs were identified in 39 patients. Two-thirds of patients had solitary brain AVMs, whereas 33% of patients harbored at least two lesions (range: 2-16). Brain AVMs of the supratentorial cerebral hemispheres comprised 83% of all lesions, whereas infratentorial lesions accounted for only 17%. Of the 55 brain AVMs assigned Spetzler-Martin grading, the majority of patients were Grade 1 (73%), and 23% and 4% were Grades 2 and 3, respectively. Patients were treated with surgery alone (51%), embolization alone (6%), embolization followed by surgery (9%), stereotactic radiosurgery (11%), stereotactic radiosurgery followed by surgery (3%), or observation (20%). Of patients who underwent genetic analysis, 62% possessed mutations in ENG (HHT type 1), whereas 38% had mutations in ACVRL1 (HHT type 2). This robust patient cohort of brain AVMs in 39 patients with HHT advances the collective understanding of this disease's varied presentation, diagnostic workup, genetic underpinnings, and available treatment options.

Neurovascular patterning cues and implications for central and peripheral neurological disease.

The highly branched nervous and vascular systems run along parallel trajectories throughout the human body. This stereotyped pattern of branching shared by the nervous and vascular systems stems from a common reliance on specific cues critical to both neurogenesis and angiogenesis. Continually emerging evidence supports the notion of later-evolving vascular networks co-opting neural molecular mechanisms to ensure close proximity and adequate delivery of oxygen and nutrients to nervous tissue. As our understanding of these biologic pathways and their phenotypic manifestations continues to advance, identification of where pathways go awry will provide critical insight into central and peripheral nervous system pathology.