Utility of Anterior Atlantodens Interval Widening on Cervical Spine CT for Assessing Transverse Atlantal Ligament Injury.

STUDY DESIGN: Retrospective, cross-sectional. OBJECTIVES: To identify trauma patients with confirmed tears of the transverse atlantal ligament on cervical MRI and measure several parameters of atlanto-axial alignment on cervical CT, including the anterior atlantodens interval, to determine which method is most sensitive in predicting transverse atlantal ligament injury. METHODS: Adult trauma patients who suffered a transverse atlantal ligament tear on cervical MRI were identified retrospectively. The cervical CT and MRI exams for these patients were reviewed for the following: anterior and lateral atlantodens interval widening, lateral C1 mass offset, C1-C2 rotatory subluxation, and transverse atlantal ligament injuries on cervical MRI. RESULTS: Twenty-six patients were identified with a tear of the transverse atlantal ligament on cervical MRI. Twelve percent of these patients demonstrated an anterior dens interval measuring greater than 2 mm, 26% of patients demonstrated lateral mass offset of C1 on C2 (average offset of 2.4 mm), 18% of patients demonstrated an asymmetry greater than 1 mm between the left and right lateral atlantodens interval, and one patient demonstrated atlanto-axial rotation measuring greater than 20%. Ten patients had an accompanying C1 burst fracture and eight patients had a C2 fracture. One patient demonstrated widening of the atlanto-occipital joint space greater than 2 mm indicative of craniocervical dissociation injury. CONCLUSIONS: An anterior atlantodens interval measuring greater than 2 mm is an unreliable methodology to screen trauma patients for transverse altantal ligament injuries and atlanto-axial instability. Moreover, C1 lateral mass offset, lateral atlantodens asymmetry, and atlanto-axial rotation were all poor predictors of transverse atlantal ligament tears.

Post-Transplant Lymphoproliferative Disorder: A Rare Case of CNS Involvement following Renal Transplant.

BACKGROUND: Post-transplant lymphoproliferative disorders (PTLD) are rare immunosuppression complications affecting 5% of transplant patients. Isolated central nervous system (CNS)-PTLD without nodal or extra-nodal organ involvement is rarely reported and is difficult to diagnose due to the non-specific clinical manifestations and imaging features overlapping with other common CNS lesions. CASE PRESENTATION: We present a case of a 72-year-old female subjected to a renal transplant 11 years ago with progressively worsening headaches and confusion. Imaging revealed vasogenic edema in the left frontal and bilateral temporal lobes. She was subjected to a craniotomy and excisional biopsy to obtain tissue for diagnostic and therapeutic interventions. Pathology examination showed atypical EBV-positive lymphoplasmacytic infiltrate, consistent with Polymorphic type PTLD. CONCLUSIONS: Patients diagnosed with PTLD need to have close monitoring of immunosuppressive medications while in the hospital. Early diagnosis is essential for patient survival with PTLD, as their health can deteriorate fast.

Impact of Type of Treatment Center and Access to Care on Mortality and Survival for Skull Base Chordoma and Chondrosarcoma.

Introduction In adults with skull base chordoma or chondrosarcoma, the impact of treatment center and access to care have not been well described in regard to perioperative mortality and survival. Methods A query of the National Cancer Database (NCDB) and review of 1,102 adults-488 with chordomas and 614 with chondrosarcomas-was performed. The Kaplan-Meier's product limit method and chi-square analysis, respectively, assessed overall survival and 30-day (30D) and 90-day (90D) mortalities. Results For 925 patients who had surgery and available mortality data, the 30D and 90D mortality rates were 0.9 and 1.5%. Lower education level ( p = 0.0185) and treatment at a nonacademic facility ( p = 0.016) were associated with increased risk of 90-day mortality. Median follow-up was 52 months and analysis was dichotomized by histology. For those with skull base chordoma, patients from a larger metro size ( p = 0.002), age below the median 52 years ( p ≤ 0.001), and private insurance (<0.001) were associated with prolonged survival, whereas for skull base chondrosarcoma, the factors were treatment at an academic medical center ( p = 0.001), high-volume center ( p = 0.007), age below the median 52 years ( p ≤ 0.001), higher income ( p = 0.043), higher education ( p = 0.017), and private insurance ( p ≤ 0.001). Comparing high-, medium-, and low-volume centers, high-volume centers were most likely to be academic, deliver radiotherapy, escalate doses >70 Gy, and utilize proton radiotherapy consistent across both disease subsets. Conclusion Higher educational attainment and treatment at an academic facility were associated with decreased 90D mortality for patients with skull base chordoma and chondrosarcoma. For those with skull base chordoma, larger metro size, younger age, and private insurance were associated with prolonged survival; for those with chondrosarcoma, it was treatment at a high-volume or academic medical center, younger age, higher income or education, and private insurance.

Disease Control after Radiotherapy for Adult Craniopharyngioma: Clinical Outcomes from a Large Single-Institution Series.

PURPOSE: To report disease control and treatment-related side effects among adult patients with craniopharyngioma treated with radiotherapy. METHODS: We performed a single-institution review of adult patients (> 21 years old) with craniopharyngioma treated with radiotherapy either definitively or postoperatively for gross residual disease. We report disease control, survival, and radiotherapy-related side effects. RESULTS: A total of 49 adult patients with craniopharyngioma were included, 27 of whom were treated at initial presentation and 22 for recurrent disease following initial surgery and observation. Overall, 77% received radiotherapy postoperatively (either after primary surgery or surgery for recurrence). With a median clinical and radiographic follow-up of 4.2 (range, 0.4-21.6) years and 3.0 (range, 0-21.5) years, the 5- and 10-year local control rates were 100 and 94%, respectively. The 5- and 10-year overall survival rates were 80 and 66%, respectively. Eleven percent of patients experienced grade 2 vision deterioration and 18% suffered grade 2 endocrinopathies following radiotherapy. CONCLUSIONS: Radiotherapy provides excellent disease control with acceptable toxicity among adult patients with craniopharyngioma. These data support the use of fractionated radiotherapy in adult patients with recurrent or gross residual disease after surgery. For inoperable patients or those with moderate or high surgical risk to neurologic and/or vascular structures, we advocate for limited surgical resection and postoperative radiotherapy to balance optimal tumor control with tumor- and treatment-related morbidity.

Current Advances in the Management of Adult Craniopharyngiomas.

Craniopharyngiomas (CPs) are slow growing, histologically benign intracranial tumors located in the sellar-suprasellar region. Although known to have low mortality, their location and relationship to the adjacent neural structures results in patients having significant neurologic, endocrine, and visual comorbidities. The invasive nature of this tumor makes complete resection a challenge and contributes to its recurrence. Additionally, these tumors are bimodally distributed, being treated with surgery, and are followed by other adjuncts, such as focused radiation therapy, e.g., Gamma knife. Advances in surgical techniques, imaging tools, and instrumentations have resulted in the evolution of surgery using endoscopic techniques, with residual components being treated by radiotherapy to target the residual tumor. Advances in molecular biology have elucidated the main pathways involved in tumor development and recurrence, but presently, no other treatments are offered to patients, besides surgery, radiation, and endocrine management, as the disease and tumor evolve. We review the contemporary management of these tumors, from the evolution of surgical treatments, utilizing standard open microscopic approaches to the more recent endoscopic surgery, and discuss the current recommendations for care of these patients. We discuss the developments in radiation therapy, such as radiosurgery, being used as treatment strategies for craniopharyngioma, highlighting their beneficial effects on tumor resections while decreasing the rates of adverse outcomes. We also outline the recent chemotherapy modalities, which help control tumor growth, and the immune landscape on craniopharyngiomas that allow the development of novel immunotherapies.

Radiologic utility of the Gehweiler and AO spine classification systems for C1 Trauma: A retrospective review from a Level I trauma center.

Objective: The purpose of our study was to identify adult trauma patients with an acute C1 burst fracture, evaluate for concomitant transverse atlantal ligament (TAL) injury, and apply the modified Gehweiler and AO spine classification systems to determine the utility of these classification systems in accurately defining C1 trauma. Materials and Methods: Adult trauma patients with an acute C1 fracture were identified retrospectively using Nuance mPower software. The C1 fracture was described based on whether the fracture involved the anterior arch, posterior arch, lateral mass, medial tubercle, and/or transverse process. If follow-up cervical magnetic resonance imaging (MRI) was performed, the presence and location of an associated TAL injury was recorded. The anatomic location of the C1 burst fracture and TAL injury, if present, were compared with the descriptive classification systems outlined by Gehweiler/Dickman (modified) and the AO Spine society. Any additional osseous trauma of the skull base and C1-C2 was also recorded along with relevant clinical history and management. Results: Thirty-nine patients were identified with an acute C1 burst fracture on cervical computed tomography (CT) with seventy-seven percent of patients undergoing follow-up cervical MRI. Observed fracture patterns were divided into five distinct types based on CT findings and further subdivided based on the integrity of the transverse altantal ligament on MRI. TAL tears were observed exclusively in type 3 fractures (anterior and posterior arch fractures) and type 4 fractures (anterior arch, posterior arch, and lateral mass fractures). The modified Gehweiler classification system failed to accurately describe the anatomic location of the C1 fracture in forty-four percent of patients, whereas the AO spine was too broad and failed to accurately describe fracture location in our cohort. Conclusions: The Gehweiler and AO spine classifications demonstrated significant shortcomings in the accurate description of patients with C1 trauma. Whereas the Gehweiler system did not accurately describe the anatomic location of the various C1 fractures, the AO spine system was too broad and failed to radiologically classify fracture location. Moreover, there was a high number of patients with AO spine type B injuries without atlantoaxial translation that nevertheless required C1-C2 fusion for atlantoaxial instability. We suggest the need for an updated classification system that takes into account both the CT (fracture location) and MRI (TAL integrity) appearance of C1 trauma. An updated classification strategy will offer a radiologic standardization of C1 trauma that will aid in future research studies and help optimize patient management.

A Rare Case of Life-Threatening Multicompartmental Spontaneous Intracranial Hemorrhage From a Grade 1 Convexity Meningioma.

Meningiomas are slowly growing benign tumors. The incidence of hemorrhage associated with intracranial meningiomas is in the 0.5%-2.4% range. However, intracranial meningiomas with hemorrhagic presentation are associated with higher rates of overall major morbidity (36%) and mortality (21.1%). We report a case of a convexity meningioma presenting with intraparenchymal hematoma and bilateral acute subdural hematomas (SDH) in a comatose patient (Glasgow Coma Scale (GCS) score: 7) who had a history of recurrent episodes of headaches over the past few months. Hemorrhagic presentation of a meningioma is a rare but potentially devastating event. Early recognition of the potential underlying meningioma as a cause of bleeding followed by rapid appropriate additional imaging is crucial to direct treatment plans to achieve the best outcome.

Falx Cerebelli Meningioma: Case Report of a Rarely Occurring Tumor, Management Nuances, and Literature Review.

The falx cerebelli is a small crescent fold of dura mater that is attached to the internal occipital crest and projects forward into the posterior cerebellar notch between the cerebellar hemispheres. We report a rare case of a 61-year-old female who presented with a 1-month history of headache and gait instability. Imaging findings were suggestive of a meningioma arising from the falx cerebelli. Complete surgical resection was achieved with a standard posterior fossa midline approach. Duraplasty was performed using animal allograft dura (Duraguard) and additional layers of oxidized cellulose preparation (Surgicel), fibrin sealant, and nonsuturable collagen matrix (Duragen) were utilized to reduce the risk of a cerebrospinal fluid leak. Pathology confirmed a World Health Organization (WHO) grade-I meningioma. Postoperatively, patient with asymptomatic thrombosis of the left transverse/sigmoid sinuses and later with a pseudomeningocele managed with a lumbar drain. To our knowledge, this is the second documented case in the literature. We discuss intraoperative nuances and unique aspects in the postoperative care and management of these patients.

Current Management and Image Review of Skull Base Chordoma: What the Radiologist Needs to Know.

Chordomas of the skull-base are typically slow-growing, notochord-derived tumors that most commonly originate along the clivus. Skull base chordoma is treated with surgery and radiotherapy. Local recurrence approaches 50% at 10 years. Radiologists play a critical role in diagnosis, treatment planning, and follow-up. Surgeons and radiation oncologists rely on radiologists for pre-operative delineation of tumor and adjacent anatomy, identification of post-treatment changes and disease recurrence, and radiation treatment effects. This review provides an overview of clinical characteristics, surgical anatomy, indications for radiotherapy, identification of treatment complications, and patterns of disease recurrence for radiologists to provide value in the management of these lesions.

Microsurgical Resection of a Parasellar Meningioma Invading the Cavernous Sinus, Bone, and Optic Canal: 2-Dimensional Operative Video.

Parasellar meningiomas, regardless of the initial origin, frequently involve the optic canal and cavernous sinus, leading to visual loss and ocular movement dysfunction.1 Hyperostotic bony invasion to the surrounding skull base is common.2 Visual acuity prognosis can be improved by surgical resection through different technical maneuvers, including opening the optic canal and the preservation of the visual apparatus vascular supply by developing the intra-arachnoidal dissection planes.1,3,4 To achieve radical dissection and reduce recurrences, the extradural invaded bone is thoroughly resected. Likewise, the soft and nonadherent invasive tumor around the cavernous carotid can be extensively removed.1,3,4 New or worsening of the ophthalmoplegia is frequent after cavernous sinus exploration and tumor resection. However, it is usually temporary and improves progressively in 80% of patients.1,3,4 Safely approaching these lesions requires extensive anatomic knowledge through cadaveric dissection and training, allowing us to achieve tumor control, preserve or improve visual function, and avoid or delay irradiation therapy.1,3,4 Pituitary function preservation is also possible in many patients through surgery, which is at higher risk with irradiation. We demonstrate the technical maneuvers through a case of a 49-yr-old woman who presented with a parasellar meningioma involving the cavernous sinus, both optic canals, and extensive bony invasion. The patient consented to the surgery and publication of her images. Image at 1:14 and middle and right image at 1:31 reprinted with permission from Al-Mefty O, Operative Atlas of Meningiomas. Vol 1, ©LWW, 1998. Left image at 1:31 reprinted from Arnautovic et al,5 by permission from JNSPG.

Halo vest immobilization - an institutional review of safety in acute cervical spine injury from 2013 to 2017.

Objective: Halo vest immobilization (HVI) remains an important treatment option for occipital-cervical injuries. It provides the surgeon with a safe and effective medical treatment options for challenging patients. The aim of this study was to evaluate the safety of HVI in these patients.Methods: This retrospective study identified adult patients treated with Halo vests immobilization (HVI) for acute cervical spine injury at our metropolitan level 1 trauma center from 2013 to 2017. This heterogenous cohort included 67 consecutive patients with acute cervical spine injury secondary to trauma or iatrogenic injury following surgical intervention with a mean age of 52 and a mean injury severity score (ISS) of 18. Forty-six percent of patients were treated with HVI as an adjunct therapy to surgical fixation (both short- and long-term immobilization), 45% of patients were treated with HVI as a primary medical treatment, and 9% of patients were treated with HVI instead of failed conservative medical treatment, such as cervical braces. Results: Pneumonia during the initial hospital stay was the most common complication (25%), followed by the correction of loose pins (22%) and pin site infections (18%). Overall, 51% of patients experienced at least one of these complications. There were significant associations between low initial GCS scores and the development of pneumonia (p < 0.001), high ISS scores and the development of pneumonia (p < 0.01), and duration of HVI and the occurrence of loose pins (p < 0.05). Four patients initially treated with HVI as primary medical treatment was converted to surgical treatment due to an intolerance of HVI or non-healing injuries.Conclusions:The HVI is a safe and effective treatment modality in a subset of patients with complex cervical junction and subaxial cervical spine pathology.

Proton therapy for adult medulloblastoma: Acute toxicity and disease control outcomes.

PURPOSE: We report disease control, survival outcomes, and treatment-related toxicity among adult medulloblastoma patients who received proton craniospinal irradiation (CSI) as part of multimodality therapy. METHODS: We reviewed 20 adults with medulloblastoma (≥ 22 years old) who received postoperative proton CSI ± chemotherapy between 2008 and 2020. Patient, disease, and treatment details and prospectively obtained patient-reported acute CSI toxicities were collected. Acute hematologic data were analyzed. RESULTS: Median age at diagnosis was 27 years; 45% of patients had high-risk disease; 75% received chemotherapy, most (65%) after CSI. Eight (40%) patients received concurrent vincristine with radiotherapy. Median CSI dose was 36GyE with a median tumor bed boost of 54GyE. Median duration of radiotherapy was 44 days. No acute ≥ grade 3 gastrointestinal or hematologic toxicities attributable to CSI occurred. Grade 2 nausea and vomiting affected 25% and 5% of patients, respectively, while 36% developed acute grade 2 hematologic toxicity (36% grade 2 leukopenia and 7% grade 2 neutropenia). Those receiving concurrent chemotherapy with CSI had a 38% rate of grade 2 hematologic toxicity compared to 33% among those not receiving concurrent chemotherapy. Among patients receiving adjuvant chemotherapy (n = 13), 100% completed ≥ 4 cycles and 85% completed all planned cycles. With a median follow-up of 3.1 years, 4-year actuarial local control, disease-free survival, and overall survival rates were 90%, 90%, and 95%, respectively. CONCLUSIONS: Proton CSI in adult medulloblastoma patients is very well tolerated and shows promising disease control and survival outcomes. These data support the standard use of proton CSI for adult medulloblastoma.

Radical Resection of a Giant Epidermoid Tumor Associated With Miniature Chordoma Utilizing a Combined Endoscopic-Microscopic Technique: 2-Dimensional Operative Video.

Epidermoid tumors arise from misplaced squamous epithelium and enlarge through the accumulation of desquamated cell debris.1 Notwithstanding the prevailing conservative attitudes to minimize morbidity, optimal treatment consists of total removal of the capsule2,3; therefore, giant and multicompartmental tumors are particularly challenging. The utilization of simultaneous endoscopic microscopic techniques by tandem endoscopic and microscopic dissection to overcome the shortcomings of both modalities, markedly enhances the ability of radical removal,4 thus eliminating or at least long-delaying inevitable recurrences with subsequent accumulated morbidity. The transmastoid approach by skeletonizing and reflexing the transverse-sigmoid sinus offers wide exposure of the cerebellopontine angle avoiding cerebellar retraction and allowing 4-hands dissection.5 The patient is a 17-yr-old male with a giant epidermoid tumor in the cerebellopontine angle, extending through the incisura. The patient underwent surgical resection with maximum pursuit of the epithelial capsule. After removing the epidermoid tumor, a miniature intra and extradural midclival tumor was encountered and removed with a proven pathology of chordoma. Patient did well postoperatively with relief of his hemifacial spasms. Patient consented for surgery and photograph publication. Image at 1:23, ©1997, O. Al-Mefty, used with permission. All rights reserved.

Spontaneous syrinx resolution in patient with Chiari I malformation: illustrative case.

BACKGROUIND: Chiari malformations include a spectrum of congenital hindbrain herniation syndromes. In patients with the most common subtype, Chiari malformation Type I, 50% to 75% develop a syrinx. The pathogenesis of syringomyelia is not well understood, with multiple theories outlined in the literature. Although the presence of a syrinx in a patient with Chiari malformation is generally accepted as an indication for surgical intervention, there are documented cases of spontaneous resolution that support a more conservative approach to management. OBSERVATIONS: The authors reported a case of spontaneous resolution of a cervical syrinx in an adult with an unchanged Chiari malformation. LESSONS: Given the possibility of spontaneous resolution over time, the authors believe a more conservative approach of observation with periodic surveillance, magnetic resonance imaging, and neurological examination should be considered in the management of a patient with a Chiari malformation and associated syringomyelia.

False Localizing Trigeminal V2 Sensory Loss in Vestibular Schwannoma.

False localizing signs involving cranial nerves are rare, even more so when involving the trigeminal nerve. Here we present the first case of trigeminal V2 sensory loss as a false localizing sign. The sensory dysfunction was caused by a large contralateral cystic vestibular schwannoma and subsequently improved after tumor resection. The clinical and radiographic features are described, and proposed mechanisms for this false localizing sign are discussed.

Is Falcine Meningioma a Diffuse Disease of the Falx? Case Series and Analysis of a "Grade Zero" Resection.

BACKGROUND: Falcine meningiomas have unique characteristics including their high rates of recurrence, association with high grade pathology, increased male prevalence, and potential for diffuse involvement of the falx. OBJECTIVE: To address these issues in a substantial series of falcine meningiomas and report on the impact of extent of resection for this distinct meningioma entity. METHODS: Retrospective analysis of characteristics and outcomes of 59 falcine meningioma patients who underwent surgery with the senior author. A "Grade Zero" category was used when an additional resection margin of 2 to 3 cm from the tumor insertion was achieved. RESULTS: For de novo falcine meningiomas, gross total resection (GTR) was associated with significantly decreased recurrence incidence compared with subtotal resection (P ≤ .0001). For recurrent falcine meningiomas, median progression-free survival (PFS) was significantly improved for GTR cases (37 mo vs 12 mo; P = .017, hazard ratio (HR) .243 (.077-.774)). "Grade Zero" resection demonstrated excellent durability for both de novo and recurrent cases, and PFS was significantly improved with "Grade Zero" resection for recurrent cases (P = .003, HR 1.544 (1.156-2.062)). The PFS benefit of "Grade Zero" resection did not achieve statistical significance over Simpson grade 1 during the limited follow-up period (mean 2.8 yr) for these groups. CONCLUSION: The recurrence of falcine meningiomas is related to the diffuse presence of tumor between the leaflets of the falx. Increased extent of resection including, when possible, a clear margin of falx surrounding the tumor base was associated with the best long-term outcomes in our series, particularly for recurrent tumors.

Combined Posterior-Anterior Interbody Fusion in the Management of Traumatic Lumbosacral Dissociation: A Case Report and Review of Literature.

Traumatic lumbosacral dissociation is a unique, but well-documented, phenomenon that generally stems from high-energy impact injuries to the lower lumbar spine. Patients typically present with complicated and multisystem injuries with wide-ranging neurological deficits below the level of trauma. This presents stark challenges regarding the diagnosis, management, and surgical correction technique utilized. In this study, we present the case of a 21-year-old, morbidly obese, male patient that presented after a traumatic motor vehicle accident with L5-S1 lumbosacroiliac dissociation, cauda equina syndrome, and left lower extremity monoplegia. The degree of disruption warranted a 360° approach, we opted for an anterior lumbar interbody fusion followed by a posterior, lumbar interbody, short segment fusion. We review the case and relevant literature of similar lumbosacral dissociation studies with their management options and outcomes. Due to the rare nature of these devastating injuries, there remains wide variability in their management, with a combination of open anterior and posterior approaches resulting in variable long-term outcomes. The management of these rare injuries will require appropriate consideration of the patient's unique etiology, coexisting injuries, and radiological imaging in deciding surgical stabilization techniques.

Spontaneous Cerebral Spinal Fluid Rhinorrhea with Meningocele Secondary to Congenital Foramen Rotundum Defect: A Case Report and Review of Literature.

Spontaneous cerebrospinal fluid (CSF) rhinorrhea is a rare occurrence. We present a case of spontaneous CSF rhinorrhea in a 57-year-old patient secondary to a sphenoid osseous defect involving the foramen rotundum and maxillary nerve with an associated arachnoid cyst and meningocele compressing the maxillary nerve. The location of the defect made correction amenable to an open skull-based approach. To the best of our knowledge, this is the first reported case of a spontaneous meningocele herniating into the sphenoid osseous defect through the medial aspect of the foramen rotundum. Early detection of these defects, open or endoscopic approaches and definitive treatment by closing the defect can result in excellent outcomes.

A case of a facial nerve venous malformation presenting with crocodile tear syndrome.

BACKGROUND: Crocodile tears syndrome, also known as Bogorad syndrome, is characterized by lacrimation secondary to olfactory and gustatory stimuli and mastication. Crocodile tear syndrome is typically encountered as an uncommon complication of Bell's palsy and usually occurs during the recovery phase of the disease course. CASE DESCRIPTION: We present a case of a 39-year-old male who presented with facial paralysis with ipsilateral crocodile tear syndrome caused by a slow flow venous malformation of the petrous bone and facial nerve. CONCLUSION: We present a case of crocodile tear syndrome caused by a facial nerve venous malformation. To the best of our knowledge, this is the only case reported in literature.