Endovascular Thrombectomy Treatment Effect in Direct vs Transferred Patients With Large Ischemic Strokes: A Prespecified Analysis of the SELECT2 Trial.

Importance: Patients with large ischemic core stroke have poor clinical outcomes and are frequently not considered for interfacility transfer for endovascular thrombectomy (EVT). Objective: To assess EVT treatment effects in transferred vs directly presenting patients and to evaluate the association between transfer times and neuroimaging changes with EVT clinical outcomes. Design, Setting, and Participants: This prespecified secondary analysis of the SELECT2 trial, which evaluated EVT vs medical management (MM) in patients with large ischemic stroke, evaluated adults aged 18 to 85 years with acute ischemic stroke due to occlusion of the internal carotid or middle cerebral artery (M1 segment) as well as an Alberta Stroke Program Early CT Score (ASPECTS) of 3 to 5, core of 50 mL or greater on imaging, or both. Patients were enrolled between October 2019 and September 2022 from 31 EVT-capable centers in the US, Canada, Europe, Australia, and New Zealand. Data were analyzed from August 2023 to January 2024. Interventions: EVT vs MM. Main Outcomes and Measures: Functional outcome, defined as modified Rankin Scale (mRS) score at 90 days with blinded adjudication. Results: A total of 958 patients were screened and 606 patients were excluded. Of 352 enrolled patients, 145 (41.2%) were female, and the median (IQR) age was 66.5 (58-75) years. A total of 211 patients (59.9%) were transfers, while 141 (40.1%) presented directly. The median (IQR) transfer time was 178 (136-230) minutes. The median (IQR) ASPECTS decreased from the referring hospital (5 [4-7]) to an EVT-capable center (4 [3-5]). Thrombectomy treatment effect was observed in both directly presenting patients (adjusted generalized odds ratio [OR], 2.01; 95% CI, 1.42-2.86) and transferred patients (adjusted generalized OR, 1.50; 95% CI, 1.11-2.03) without heterogeneity (P for interaction = .14). Treatment effect point estimates favored EVT among 82 transferred patients with a referral hospital ASPECTS of 5 or less (44 received EVT; adjusted generalized OR, 1.52; 95% CI, 0.89-2.58). ASPECTS loss was associated with numerically worse EVT outcomes (adjusted generalized OR per 1-ASPECTS point loss, 0.89; 95% CI, 0.77-1.02). EVT treatment effect estimates were lower in patients with transfer times of 3 hours or more (adjusted generalized OR, 1.15; 95% CI, 0.73-1.80). Conclusions and Relevance: Both directly presenting and transferred patients with large ischemic stroke in the SELECT2 trial benefited from EVT, including those with low ASPECTS at referring hospitals. However, the association of EVT with better functional outcomes was numerically better in patients presenting directly to EVT-capable centers. Prolonged transfer times and evolution of ischemic change were associated with worse EVT outcomes. These findings emphasize the need for rapid identification of patients suitable for transfer and expedited transport. Trial Registration: ClinicalTrials.gov Identifier: NCT03876457.

Mediation of Successful Reperfusion Effect through Infarct Growth and Cerebral Edema: A Pooled, Patient-Level Analysis of EXTEND-IA Trials and SELECT Prospective Cohort.

OBJECTIVE: Reperfusion therapy is highly beneficial for ischemic stroke. Reduction in both infarct growth and edema are plausible mediators of clinical benefit with reperfusion. We aimed to quantify these mediators and their interrelationship. METHODS: In a pooled, patient-level analysis of the EXTEND-IA trials and SELECT study, we used a mediation analysis framework to quantify infarct growth and cerebral edema (midline shift) mediation effect on successful reperfusion (modified Treatment in Cerebral Ischemia ≥ 2b) association with functional outcome (modified Rankin Scale distribution). Furthermore, we evaluated an additional pathway to the original hypothesis, where infarct growth mediated successful reperfusion effect on midline shift. RESULTS: A total 542 of 665 (81.5%) eligible patients achieved successful reperfusion. Baseline clinical and imaging characteristics were largely similar between those achieving successful versus unsuccessful reperfusion. Median infarct growth was 12.3ml (interquartile range [IQR] = 1.8-48.4), and median midline shift was 0mm (IQR = 0-2.2). Of 249 (37%) demonstrating a midline shift of ≥1mm, median shift was 2.75mm (IQR = 1.89-4.21). Successful reperfusion was associated with reductions in both predefined mediators, infarct growth (ß = -1.19, 95% confidence interval [CI] = -1.51 to -0.88, p < 0.001) and midline shift (adjusted odds ratio = 0.36, 95% CI = 0.23-0.57, p < 0.001). Successful reperfusion association with improved functional outcome (adjusted common odds ratio [acOR] = 2.68, 95% CI = 1.86-3.88, p < 0.001) became insignificant (acOR = 1.39, 95% CI = 0.95-2.04, p = 0.094) when infarct growth and midline shift were added to the regression model. Infarct growth and midline shift explained 45% and 34% of successful reperfusion effect, respectively. Analysis considering an alternative hypothesis demonstrated consistent results. INTERPRETATION: In this mediation analysis from a pooled, patient-level cohort, a significant proportion (~80%) of successful reperfusion effect on functional outcome was mediated through reduction in infarct growth and cerebral edema. Further studies are required to confirm our findings, detect additional mediators to explain successful reperfusion residual effect, and identify novel therapeutic targets to further enhance reperfusion benefits. ANN NEUROL 2023;93:793-804.

Predictors of In-Hospital Mortality and Home Discharge in Patients with Aneurysmal Subarachnoid Hemorrhage: A 4-Year Retrospective Analysis.

BACKGROUND: Factors associated with discharge disposition and mortality following aneurysmal subarachnoid hemorrhage (aSAH) are not well-characterized. We used a national all-payer database to identify factors associated with home discharge and in-hospital mortality. METHODS: The National Inpatient Sample was queried for patients with aSAH within a 4-year range. Weighted multivariable logistic regression models were constructed and adjusted for age, sex, race, household income, insurance status, comorbidity burden, National Inpatient Sample SAH Severity Score, disease severity, treatment modality, in-hospital complications, and hospital characteristics (size, teaching status, and region). RESULTS: Our sample included 37,965 patients: 33,605 were discharged alive and 14,350 were discharged home. Black patients had lower odds of in-hospital mortality compared with White patients (adjusted odds ratio [aOR] = 0.67, 95% confidence interval [CI] 0.52-0.86, p = 0.002). Compared with patients with private insurance, those with Medicare were less likely to have a home discharge (aOR = 0.58, 95% CI 0.46-0.74, p < 0.001), whereas those with self-pay (aOR = 2.97, 95% CI 2.29-3.86, p < 0.001) and no charge (aOR = 3.21, 95% CI 1.57-6.55, p = 0.001) were more likely to have a home discharge. Household income percentile was not associated with discharge disposition or in-hospital mortality. Paradoxically, increased number of Elixhauser comorbidities was associated with significantly lower odds of in-hospital mortality. CONCLUSIONS: We demonstrate independent associations with hospital characteristics, patient characteristics, and treatment characteristics as related to discharge disposition and in-hospital mortality following aSAH, adjusted for disease severity.

Functional Outcomes and Postoperative Cerebral Venous Sinus Thrombosis after Translabyrinthine Approach for Vestibular Schwannoma Resection: A Radiographic Demonstration of Anatomic Predictors.

Introduction While regarded as an effective surgical approach to vestibular schwannoma (VS) resection, the translabyrinthine (TL) approach is not without complications. It has been postulated that postoperative cerebral venous sinus thrombosis (pCVST) may occur as a result of injury and manipulation during surgery. Our objective was to identify radiologic, surgical, and patient-specific risk factors that may be associated with pCVST. Methods The Institutional Review Board (IRB) approval was obtained and the medical records of adult patients with VS who underwent TL craniectomy at University Hospitals Cleveland Medical Center between 2009 and 2019 were reviewed. Demographic data, radiographic measurements, and tumor characteristics were collected. Outcomes assessed included pCVST and the modified Rankin score (mRS). Results Sixty-one patients ultimately met inclusion criteria for the study. Ten patients demonstrated radiographic evidence of thrombus. Patients who developed pCVST demonstrated shorter internal auditory canal (IAC) to sinus distance (mean: 22.5 vs. 25.0 mm, p = 0.044) and significantly smaller petrous angles (mean: 26.3 vs. 32.7 degrees, p = 0.0045). Patients with good mRS scores (<3) appeared also to have higher mean petrous angles (32.5 vs. 26.8, p = 0.016). Koos' grading and tumor size, in our study, were not associated with thrombosis. Conclusion More acute petrous angle and shorter IAC to sinus distance are objective anatomic variables associated with pCVST in TL surgical approaches.

Vision Deterioration and Hydrocephalus: Rare Presentations of Vestibular Schwannoma and Evolution of Management.

Introduction The incidence of vestibular schwannoma is reported as 12 to 54 new cases per million per year, increasing over time. These patients usually present with unilateral sensorineural hearing loss, tinnitus, or vertigo. Rarely, these patients present with symptoms of hydrocephalus or vision changes. Objective The study aimed to evaluate the surgical management of vestibular schwannoma at a single institution and to identify factors that may contribute to hydrocephalus, papilledema, and the need for pre-resection diversion of cerebrospinal fluid. Patients and Methods A retrospective review examining the data of 203 patients with vestibular schwannoma managed with surgical resection from May 2008 to May 2020. We stratified patients into five different groups to analyze: tumors with a diameter of ≥40 mm, clinical evidence of hydrocephalus, and of papilledema, and patients who underwent pre-resection cerebrospinal fluid (CSF) diversion. Results From May 2008 to May 2020, 203 patients were treated with surgical resection. Patients with tumors ≥40 mm were more likely to present with visual symptoms ( p < 0.001). Presentation with hydrocephalus was associated with larger tumor size ( p < 0.001) as well as concomitant visual symptoms and papilledema ( p < 0.001). Patients with visual symptoms presented at a younger age ( p = 0.002) and with larger tumors ( p < 0.001). Conclusion This case series highlights the rare presentation of vision changes and hydrocephalus in patients with vestibular schwannoma. We recommend urgent CSF diversion for patients with visual symptoms and hydrocephalus, followed by definitive resection. Further, vision may still deteriorate even after CSF diversion and tumor resection.

Bypassing the intensive care unit for patients with acute ischemic stroke secondary to large-vessel occlusion.

OBJECTIVE: Endovascular mechanical thrombectomy is safe and effective for the treatment of acute ischemic stroke (AIS) due to large-vessel occlusion (LVO). Still, despite high rates of procedural success, it is routine practice to uniformly admit postthrombectomy patients to an intensive care unit (ICU) for postoperative observation. Predictors of ICU criteria and care requirements in the postmechanical thrombectomy ischemic stroke patient population are lacking. The goal of the present study is to identify risk factors associated with requiring ICU-level intervention following mechanical thrombectomy. METHODS: The authors retrospectively analyzed data from 245 patients undergoing thrombectomy for AIS from anterior circulation LVO at a comprehensive stroke and tertiary care center from January 2015 to March 2020. Clinical variables that predicted the need for critical care intervention were identified and compared. The performance of a binary classification test constructed from these predictive variables was also evaluated using a validation cohort. RESULTS: Seventy-six patients (31%) required critical care interventions. A recanalization grade lower than modified Thrombolysis in Cerebral Infarction (mTICI) scale grade 2B (odds ratio [OR] 3.625, p = 0.001), Alberta Stroke Program Early Computed Tomography Score (ASPECTS) < 8 (OR 3.643, p < 0.001), and presence of hyperdensity on postprocedure cone-beam CT (OR 2.485, p = 0.005) were significantly associated with the need for postthrombectomy critical care intervention. When applied to a validation cohort, a clearance classification scheme using these three variables demonstrated high positive predictive value (0.88). CONCLUSIONS: A recanalization grade lower than mTICI 2B, ASPECTS < 8, and postprocedure hyperdensity on cone-beam CT were shown to be independent predictors of requiring ICU-level care. Routine admission to ICU-level care can be costly and confer increased risk for hospital-acquired conditions. Safely and reliably identifying low-risk patients has the potential for cost savings, value-based care, and decreasing hospital-acquired conditions.

En Plaque Meningiomas: A Narrative Review.

Background En plaque meningiomas are a rare subtype of meningiomas that are frequently encountered in the spheno-orbital region. Characterized by a hyperostotic and dural invasive architecture, these tumors present unique diagnostic and treatment considerations. Objective The authors conduct a narrative literature review of clinical reports of en plaque meningiomas to summarize the epidemiology, clinical presentation, diagnostic criteria, and treatment considerations in treating en plaque meningiomas. Additionally, the authors present a case from their own experience to illustrate its complexity and unique features. Methods A literature search was conducted using the MEDLINE database using the following terminology in various combinations: meningioma , meningeal neoplasms, en plaque , skull base , spheno-orbital, and sphenoid wing . Only literature published in English between 1938 and 2018 was reviewed. All case series were specifically reviewed for sufficient data on treatment outcomes, and all literature was analyzed for reports of misdiagnosed cases. Conclusion En plaque meningiomas may present with a variety of symptoms according to their location and degree of bone invasion, requiring a careful diagnostic and treatment approach. While early and aggressive surgical resection is generally accepted as the optimal goal of treatment, these lesions require an individualized approach, with further investigation needed regarding the role of new therapies.

Management of giant prolactinoma causing craniocervical instability: illustrative case.

BACKGROUND: Giant prolactinomas (>4 cm) are a rare entity, constituting less than 1% of all pituitary tumors. Diagnosis can usually be achieved through endocrinological analysis, but biopsy may be considered when trying to differentiate between invasive nonfunctioning pituitary adenomas and primary clival tumors such as chordomas. OBSERVATIONS: The authors presented a rare case of a giant prolactinoma causing significant clival and occipital condyle erosion, which led to craniocervical instability. They provided a review of the multimodal management. Management involved medical therapy with dopamine agonists, and surgery was reserved for acute neural compression or dopamine agonist resistance, with the caveat that surgery was extremely unlikely to lead to normalization of serum prolactin in dopamine agonist-resistant tumors. LESSONS: Adjunctive surgical therapy may be necessary in cases of skull base erosion, particularly when erosion or pathological fractures involve the occipital condyles. Modern posterior occipital-cervical fusion techniques have high rates of arthrodesis and can lead to symptomatic improvement. This procedure should be considered early in the multimodal approach to giant prolactinomas because of the often dramatic response to medical therapy and potential for further craniocervical instability.

Use of Telemedicine to Improve Interfacility Communication and Aid in Triage of Patients with Intracerebral Hemorrhage: A Pilot Study.

INTRODUCTION: Over the past several years there has been a dramatic increase in the implementation of telemedicine technology to aid in the delivery of care across community, inpatient, and emergency settings. This technology has proved valuable for acute life-threatening clinical scenarios. We aimed to pilot a novel neurosurgical telemedicine program within an academic tertiary care center to assist in consultation of patients with high-grade intracranial hemorrhage (ICH) (ICH score 4, 5). METHODS: A quality improvement conceptual framework was developed. Subsequently, a process map and improvement interventions were created. Patients in community hospitals with high-grade ICH or pre-existing Do Not Resuscitate/Do Not Intubate orders with an admitting diagnosis of ICH triggered a TeleNeurosurgery consultation. Patients who met the inclusion criteria, with consent of their decision makers, were enrolled in the study. Post-encounter physician surveys were used to evaluate overall satisfaction with the implementation. RESULTS: This 18-month pilot study proved feasible, with an enrollment of 63.6% (n = 14 of 22) of patients who met criteria. All patients who were enrolled in the study and participated in TeleNeurosurgery consultation remained at the presenting facility for end-of-life care and palliative medicine consultation. Both community emergency physicians and subspecialists who performed the consultations reported satisfaction with the TeleNeurosurgery consultation process and a perceived benefit both to patients, families, and emergency medicine physicians. CONCLUSIONS: The program proved feasible and several areas in need of improvement within the health system were identified. Emergency physicians reported comfort with the process, program effectiveness, and improved access to care by implementation of this program.

Cerebral vessel anatomy as a predictor of first-pass effect in mechanical thrombectomy for emergent large-vessel occlusion.

OBJECTIVE: Mechanical thrombectomy is effective in acute ischemic stroke secondary to emergent large-vessel occlusion, but optimal efficacy is contingent on fast and complete recanalization. First-pass recanalization does not occur in the majority of patients. The authors undertook this study to determine if anatomical parameters of the intracranial vessels impact the likelihood of first-pass complete recanalization. METHODS: The authors retrospectively evaluated data obtained in 230 patients who underwent mechanical thrombectomy for acute ischemic stroke secondary to large-vessel occlusion at their institution from 2016 to 2018. Eighty-six patients were identified as having pure M1 occlusions, and 76 were included in the final analysis. The authors recorded and measured clinical and anatomical parameters and evaluated their relationships to the first-pass effect. RESULTS: The first-pass effect was achieved in 46% of the patients. When a single device was employed, aspiration thrombectomy was more effective than stent retriever thrombectomy. A larger M1 diameter (p = 0.001), decreased vessel diameter tapering between the petrous segment of the internal carotid artery (ICA) and M1 (p < 0.001), and distal collateral grading (p = 0.044) were associated with first-pass recanalization. LASSO (least absolute shrinkage and selection operator) was used to generate a predictive model for recanalization using anatomical variables. CONCLUSIONS: The authors demonstrated that a larger M1 vessel diameter, low rate of vessel diameter tapering along the course of the intracranial ICA, and distal collateral status are associated with first-pass recanalization for patients with M1 occlusions.

Guidelines for the Early Management of Patients With Acute Ischemic Stroke: 2019 Update to the 2018 Guidelines for the Early Management of Acute Ischemic Stroke: A Guideline for Healthcare Professionals From the American Heart Association/American Stroke Association.

Background and Purpose- The purpose of these guidelines is to provide an up-to-date comprehensive set of recommendations in a single document for clinicians caring for adult patients with acute arterial ischemic stroke. The intended audiences are prehospital care providers, physicians, allied health professionals, and hospital administrators. These guidelines supersede the 2013 Acute Ischemic Stroke (AIS) Guidelines and are an update of the 2018 AIS Guidelines. Methods- Members of the writing group were appointed by the American Heart Association (AHA) Stroke Council's Scientific Statements Oversight Committee, representing various areas of medical expertise. Members were not allowed to participate in discussions or to vote on topics relevant to their relations with industry. An update of the 2013 AIS Guidelines was originally published in January 2018. This guideline was approved by the AHA Science Advisory and Coordinating Committee and the AHA Executive Committee. In April 2018, a revision to these guidelines, deleting some recommendations, was published online by the AHA. The writing group was asked review the original document and revise if appropriate. In June 2018, the writing group submitted a document with minor changes and with inclusion of important newly published randomized controlled trials with >100 participants and clinical outcomes at least 90 days after AIS. The document was sent to 14 peer reviewers. The writing group evaluated the peer reviewers' comments and revised when appropriate. The current final document was approved by all members of the writing group except when relationships with industry precluded members from voting and by the governing bodies of the AHA. These guidelines use the American College of Cardiology/AHA 2015 Class of Recommendations and Level of Evidence and the new AHA guidelines format. Results- These guidelines detail prehospital care, urgent and emergency evaluation and treatment with intravenous and intra-arterial therapies, and in-hospital management, including secondary prevention measures that are appropriately instituted within the first 2 weeks. The guidelines support the overarching concept of stroke systems of care in both the prehospital and hospital settings. Conclusions- These guidelines provide general recommendations based on the currently available evidence to guide clinicians caring for adult patients with acute arterial ischemic stroke. In many instances, however, only limited data exist demonstrating the urgent need for continued research on treatment of acute ischemic stroke.

Common Data Elements for Unruptured Intracranial Aneurysms and Aneurysmal Subarachnoid Hemorrhage: Recommendations from the Working Group on Hospital Course and Acute Therapies-Proposal of a Multidisciplinary Research Group.

INTRODUCTION: The Common Data Elements (CDEs) initiative is a National Institute of Health/National Institute of Neurological Disorders and Stroke (NINDS) effort to standardize naming, definitions, data coding, and data collection for observational studies and clinical trials in major neurological disorders. A working group of experts was established to provide recommendations for Unruptured Aneurysms and Aneurysmal Subarachnoid Hemorrhage (SAH) CDEs. METHODS: This paper summarizes the recommendations of the Hospital Course and Acute Therapies after SAH working group. Consensus recommendations were developed by assessment of previously published CDEs for traumatic brain injury, stroke, and epilepsy. Unruptured aneurysm- and SAH-specific CDEs were also developed. CDEs were categorized into "core", "supplemental-highly recommended", "supplemental" and "exploratory". RESULTS: We identified and developed CDEs for Hospital Course and Acute Therapies after SAH, which included: surgical and procedure interventions; rescue therapy for delayed cerebral ischemia (DCI); neurological complications (i.e. DCI; hydrocephalus; rebleeding; seizures); intensive care unit therapies; prior and concomitant medications; electroencephalography; invasive brain monitoring; medical complications (cardiac dysfunction; pulmonary edema); palliative comfort care and end of life issues; discharge status. The CDEs can be found at the NINDS Web site that provides standardized naming, and definitions for each element, and also case report form templates, based on the CDEs. CONCLUSION: Most of the recommended Hospital Course and Acute Therapies CDEs have been newly developed. Adherence to these recommendations should facilitate data collection and data sharing in SAH research, which could improve the comparison of results across observational studies, clinical trials, and meta-analyses of individual patient data.

Relationship between middle cerebral parent artery asymmetry and middle cerebral artery aneurysm rupture risk factors.

OBJECTIVE: The exact pathophysiological mechanisms underlying cerebral aneurysm formation remain unclear. Asymmetrical local vascular geometry may play a role in aneurysm formation and progression. The object of this study was to investigate the association between the geometric asymmetry of the middle cerebral artery (MCA) and the presence of MCA aneurysms and associated high-risk features. METHODS: Using a retrospective case-control study design, the authors examined MCA anatomy in all patients who had been diagnosed with an MCA aneurysm in the period from 2008 to 2017 at the University Hospitals Cleveland Medical Center. Geometric features of the MCA ipsilateral to MCA aneurysms were compared with those of the unaffected contralateral side (secondary control group). Then, MCA geometry was compared between patients with MCA aneurysms and patients who had undergone CTA for suspected vascular pathology but were ultimately found to have normal intracranial vasculature (primary control group). Parent vessel and aneurysm morphological parameters were measured, calculated, and compared between case and control groups. Associations between geometric parameters and high-risk aneurysm features were identified. RESULTS: The authors included 247 patients (158 cases and 89 controls) in the study. The aneurysm study group consisted of significantly more women and smokers than the primary control group. Patients with MCA bifurcation aneurysms had lower parent artery inflow angles (p = 0.01), lower parent artery tortuosity (p < 0.01), longer parent artery total length (p = 0.03), and a significantly greater length difference between ipsilateral and contralateral prebifurcation MCAs (p < 0.01) than those in primary controls. Type 2 MCA aneurysms (n = 89) were more likely to be associated with dome irregularity or a daughter sac and were more likely to have a higher cumulative total of high-risk features than type 1 MCA aneurysms (n = 69). CONCLUSIONS: Data in this study demonstrated that a greater degree of parent artery asymmetry for MCA aneurysms is associated with high-risk features. The authors also found that the presence of a long and less tortuous parent artery upstream of an MCA aneurysm is a common phenotype that is associated with a higher risk profile. The aneurysm parameters are easily measurable and are novel radiographic biomarkers for aneurysm risk assessment.

Posterior Cervical Laminoplasty for Resection Intradural Extramedullary Spinal Meningioma: 2-Dimensional Operative Video.

This operative video demonstrates a posterior cervical laminoplasty for the resection of a cervical intradural extramedullary meningioma. In addition, the natural history, treatment options, and potential complications are discussed. The patient is a 68-yr-old male who presented with left-hand grip weakness and paresthesias. Magnetic resonance imaging (MRI) demonstrated an enhancing mass that displacing the spinal cord anteriorly and causing severe flattening of the cord at C4 and C5. The patient underwent a posterior cervical laminoplasty for tumor resection. Removal of the dorsal elements with a high-speed drill was performed at C3, C4, and C5. A midline durotomy was performed and a large extra-axial intradural tumor was encountered. The tumor was resected en bloc and specimens were sent for permanent pathological analysis. The dura was closed in a watertight fashion using 6-0 Prolene sutures. The laminoplasty was performed by using titanium miniplates and screws to reconstruct the dorsal bony elements, and the wound was closed in layers using sutures. There were no complications. Final pathology was consistent with a WHO grade I meningioma. Postoperative MRI demonstrated gross total resection. The patient's perioperative course was uncomplicated and his preoperative weakness completely resolved by time of discharge.

Intratumoral Rathke's Cleft Cyst Remnants Within Craniopharyngioma, Pituitary Adenoma, Suprasellar Dermoid, and Epidermoid Cysts: A Ubiquitous Signature of Ectodermal Lineage or a Transitional Entity?

BACKGROUND: The authors present cystic epithelial masses in the suprasellar region which on histopathology revealed 4 mixed tumors having simple cuboidal epithelium of Rathke's Cleft Cyst (RCC) elements trapped within pituitary adenoma, epidermoid cyst, dermoid cyst, and papillary craniopharyngioma respectively. OBJECTIVE: To highlight the developmental theory of ectodermal continuum in the realm of suprasellar epithelial cystic lesions and examines the cardinal aspects that distinguish RCC from its confounder, ciliary craniopharyngioma. METHODS: The authors performed a medical chart review on 4 patients who had coexisting RCC with craniopharyngioma, pituitary adenoma, suprasellar dermoid, and epidermoid cysts. RESULTS: This series of unique suprasellar lesions elucidate the spectrum of cases from Rathke's cyst to other suprasellar epithelial cysts including a recently identified clinical entity called ciliary craniopharyngioma, which authors feel is a misnomer. The authors also report the first case of ruptured dermoid cyst admixed with elements of Rathke's cyst elements and xanthogranuloma in neurosurgical literature. CONCLUSION: We propose that the new entity of ciliary craniopharyngioma could be just another variant of RCC elements nested within a typical papillary or adamantinomatous lesion. Further study is warranted to understand the implications of natural history with tumors containing RCC elements.

Resection of a Thoracic Hemangioblastoma in a Patient With Von Hippel-Lindau: 3-Dimensional Operative Video.

This 3-dimensional operative video illustrates resection of a thoracic hemangioblastoma in a 30-year-old female with a history of Von Hippel-Lindau disease. The patient presented with right lower extremity numbness and flank pain. Magnetic resonance imaging (MRI) demonstrated an enhancing intradural intramedullary lesion at T 7 consistent with a hemangioblastoma. The patient underwent a thoracic laminectomy with a midline dural opening for tumor resection. This case demonstrates the principles of intradural intramedullary spinal cord tumor resection. In this particular case, internal debulking was untenable owing to the vascular nature of hemangioblastomas. The operative video demonstrates en bloc tumor removal. Postoperative MRI demonstrated gross total resection. The postoperative course was uneventful. The natural history of this disease, treatment options, and potential complications are discussed.