Bilateral STA-MCA bypass for Moyamoya angiopathy associated with severe erythrodermic psoriasis.

OBJECTIVE: We report on the uncommon association between severe erythrodermic psoriasis and moyamoya angiopathy (MMA), a progressive cerebrovascular disorder characterized by steno-occlusive changes in the circle of Willis. Concomitant moyamoya and severe erythrodermic psoriasis is a rare pathology, with unknown pathogenesis. MMA with severe erythrodermic psoriasis, even in the setting of stroke, is often managed with non-curative medical intervention alone, due to concerns for surgical instability. Here we show with appropriate surgical consideration and medical management, patients can undergo curative surgical management, and remain stroke free during follow-up. CASE REPORT: The patient, a 52-year-old female, with refractory psoriasis, presented with neurological deficits, leading to the diagnosis of bilateral moyamoya arteriopathy. Patients with these co-existing conditions have historically only been medially managed, due to concerns for surgical instability and inadequate candidacy. A comprehensive stroke workup revealed severe stenosis in the internal carotid arteries. A two-stage surgical revascularization, including right superficial temporal artery-middle cerebral artery (STA-MCA) bypass and subsequent left STA-MCA bypass, was successfully performed. Postoperatively, the patient experienced a severe psoriasis flare, requiring meticulous management to ensure post-operative surgical stability. CONCLUSIONS: With appropriate medical and surgical management, the patient was amenable for curative surgical intervention. The successful surgical intervention, following medical optimization of psoriasis, demonstrated efficacy in preventing future cerebral ischemia events in this challenging patient.

Syndrome of anterior neural stalk, vertebral abnormality, enteric duplication cyst, and diaphragmatic hernia related to persistent ventral neurenteric canal: report of two cases.

PURPOSE: Abnormalities in notochordal development can cause a range of developmental malformations, including the split notochord syndrome and split cord malformations. We describe two cases that appear related to unusual notochordal malformations, in a female and a male infant diagnosed in the early postnatal and prenatal periods, which were treated at our institution. These cases were unusual from prior cases given a shared constellation of an anterior cervicothoracic meningocele with a prominent "neural stalk," which coursed ventrally from the spinal cord into the thorax in proximity to a foregut duplication cyst. METHODS: Two patients with this unusual spinal cord anomaly were assessed clinically, and with neuroimaging and genetics studies. RESULTS: We describe common anatomical features (anterior neural stalk arising from the spinal cord, vertebral abnormality, enteric duplication cyst, and diaphragmatic hernia) that support a common etiopathogenesis and distinguish these cases. In both cases, we opted for conservative neurosurgical management in regards to the spinal cord anomaly. We proposed a preliminary theory of the embryogenesis that explains these findings related to a persistence of the ventral portion of the neurenteric canal. CONCLUSION: These cases may represent a form of spinal cord malformation due to a persistent neurenteric canal and affecting notochord development that has rarely been described. Over more than 1 year of follow-up while managed conservatively, there was no evidence of neurologic dysfunction, so far supporting a treatment strategy of observation.

Deformable MRI-Ultrasound registration using correlation-based attribute matching for brain shift correction: Accuracy and generality in multi-site data.

Intraoperative tissue deformation, known as brain shift, decreases the benefit of using preoperative images to guide neurosurgery. Non-rigid registration of preoperative magnetic resonance (MR) to intraoperative ultrasound (iUS) has been proposed as a means to compensate for brain shift. We focus on the initial registration from MR to predurotomy iUS. We present a method that builds on previous work to address the need for accuracy and generality of MR-iUS registration algorithms in multi-site clinical data. High-dimensional texture attributes were used instead of image intensities for image registration and the standard difference-based attribute matching was replaced with correlation-based attribute matching. A strategy that deals explicitly with the large field-of-view mismatch between MR and iUS images was proposed. Key parameters were optimized across independent MR-iUS brain tumor datasets acquired at 3 institutions, with a total of 43 tumor patients and 758 reference landmarks for evaluating the accuracy of the proposed algorithm. Despite differences in imaging protocols, patient demographics and landmark distributions, the algorithm is able to reduce landmark errors prior to registration in three data sets (5.37±4.27, 4.18±1.97 and 6.18±3.38 mm, respectively) to a consistently low level (2.28±0.71, 2.08±0.37 and 2.24±0.78 mm, respectively). This algorithm was tested against 15 other algorithms and it is competitive with the state-of-the-art on multiple datasets. We show that the algorithm has one of the lowest errors in all datasets (accuracy), and this is achieved while sticking to a fixed set of parameters for multi-site data (generality). In contrast, other algorithms/tools of similar performance need per-dataset parameter tuning (high accuracy but lower generality), and those that stick to fixed parameters have larger errors or inconsistent performance (generality but not the top accuracy). Landmark errors were further characterized according to brain regions and tumor types, a topic so far missing in the literature.

Type II odontoid fracture in elderly patients treated conservatively: is fracture healing the goal?

PURPOSE: Analysis of functional outcome of elderly patients with type II odontoid fractures treated conservatively in relation to their radiological outcome. METHODS: A total of 50 geriatric patients with type II odontoid fractures were treated with Aspen/Vista collars. On admission, each patient was assessed assigning ASA score, modified Rankin Scale (mRS-pre) and Charlson Comorbidity Index (CCI). From 12-15 months after treatment, functional evaluations were performed employing a second modified Rankin Scale (mRS-post) together with Neck Disability Index (NDI) and Smiley-Webster pain scale (SWPS). Radiological outcome was evaluated through dynamic cervical spine X-rays at 3 months and cervical spine CT scans 6 months after treatment. Three different conditions were identified: stable union, stable non-union and unstable non-union. Surgery was preferred whenever a fracture gap > 2 mm, an antero-posterior displacement > 5 mm, an odontoid angulation > 11° or neurological deficits occurred. RESULTS: Among the 50 patients, 24 reached a stable union, while 26 a stable non-union. Comparing the two groups, no differences in ASA (p = 0.60), CCI (p = 0.85) and mRS-pre (p = 0.14) were noted. Similarly, no differences in mRS-post (p = 0.96), SWPS (p = 0.85) and NDI (p = 0.51) were observed between patients who reached an osseous fusion and those with a stable fibrous non-union. No effects of age, sex, ASA, mRS-pre, fracture dislocation and radiological outcome were discovered on functional outcome. At logistic regression analysis, female sex and high values of CCI emerged associated with worse NDI. CONCLUSIONS: In geriatric type II odontoid fractures, pre-injury clinical status and comorbidities overcome imaging in determining post-treatment level of function. Hard collar immobilization led to a favourable functional outcome with mRS-post, NDI and SWPS values diffusely encouraging whatever a bony union or a fibrous non-union was obtained. These slides can be retrieved under Electronic Supplementary Material.

Volumetric analysis of magnetic resonance-guided focused ultrasound thalamotomy lesions.

OBJECTIVE Magnetic resonance-guided focused ultrasound (MRgFUS) thalamotomy was recently approved for use in the treatment of medication-refractory essential tremor (ET). Previous work has described lesion appearance and volume on MRI up to 6 months after treatment. Here, the authors report on the volumetric segmentation of the thalamotomy lesion and associated edema in the immediate postoperative period and 1 year following treatment, and relate these radiographic characteristics with clinical outcome. METHODS Seven patients with medication-refractory ET underwent MRgFUS thalamotomy at Brigham and Women's Hospital and were monitored clinically for 1 year posttreatment. Treatment effect was measured using the Clinical Rating Scale for Tremor (CRST). MRI was performed immediately postoperatively, 24 hours posttreatment, and at 1 year. Lesion location and the volumes of the necrotic core (zone I) and surrounding edema (cytotoxic, zone II; vasogenic, zone III) were measured on thin-slice T2-weighted images using Slicer 3D software. RESULTS Patients had significant improvement in overall CRST scores (baseline 51.4 ± 10.8 to 24.9 ± 11.0 at 1 year, p = 0.001). The most common adverse events (AEs) in the 1-month posttreatment period were transient gait disturbance (6 patients) and paresthesia (3 patients). The center of zone I immediately posttreatment was 5.61 ± 0.9 mm anterior to the posterior commissure, 14.6 ± 0.8 mm lateral to midline, and 11.0 ± 0.5 mm lateral to the border of the third ventricle on the anterior commissure-posterior commissure plane. Zone I, II, and III volumes immediately posttreatment were 0.01 ± 0.01, 0.05 ± 0.02, and 0.33 ± 0.21 cm3, respectively. These volumes increased significantly over the first 24 hours following surgery. The edema did not spread evenly, with more notable expansion in the superoinferior and lateral directions. The spread of edema inferiorly was associated with the incidence of gait disturbance. At 1 year, the remaining lesion location and size were comparable to those of zone I immediately posttreatment. Zone volumes were not associated with clinical efficacy in a statistically significant way. CONCLUSIONS MRgFUS thalamotomy demonstrates sustained clinical efficacy at 1 year for the treatment of medication-refractory ET. This technology can create accurate, predictable, and small-volume lesions that are stable over time. Instances of AEs are transient and are associated with the pattern of perilesional edema expansion. Additional analysis of a larger MRgFUS thalamotomy cohort could provide more information to maximize clinical effect and reduce the rate of long-lasting AEs.

Resection of pituitary tumors: endoscopic versus microscopic.

Transsphenoidal microscopic pituitary surgery has long been considered the gold standard in surgical treatment of pituitary tumors. Endonasal endoscopic pituitary surgery has come into prominence over the last two decades as an alternative to microscopic surgery. In this review, we use recent literature to discuss the advantages and disadvantages of each approach. Our review shows that for small intrasellar tumors, both approaches appear equally effective in experienced hands. For larger tumors with extrasellar extension, the endoscopic approach offers several advantages and may improve outcomes associated with the extent of resection and postoperative complications.

Cavernous venous malformations in and around the central nervous system. Part 1: Dural and extradural.

Cavernous-type malformations are venous lesions that occur in multiple locations throughout the body, and when present in the CNS, they have canonically been referred to as cavernomas, cavernous angiomas, and cerebral cavernous malformations. Herein all these lesions are referred to as "cavernous venous malformations" (CavVMs), which is congruent with the current International Society for the Study of Vascular Anomalies classification system. Even though histologically similar, depending on their location relative to the dura mater, these malformations can have different features. In Part 1 of this review, the authors discuss and review pertinent clinical knowledge with regard to CavVMs as influenced by anatomical location, starting with the dural and extradural malformations. They particularly emphasize dural CavVMs (including those in the cavernous sinus), orbital CavVMs, and spinal CavVMs. The genetic and histopathological features of CavVMs in these locations are reviewed, and commonalities in their presumed mechanisms of pathogenesis support the authors' conceptualization of a spectrum of a single disease entity. Illustrative cases for each subtype are presented, and the pathophysiological and genetic features linking dural and extradural to intradural CavVMs are examined. A new classification is proposed to segregate CavVMs based on the location from which they arise, which guides their natural history and treatment.

Cavernous venous malformations in and around the central nervous system. Part 2: Intradural.

Cavernous venous malformations (CavVMs) account for a spectrum of lesions with a shared pathogenesis. Their anatomical location dictates their clinical features and surgical treatment. Extradural and dura-based CavVMs were discussed in Part 1 of this review. In this part, intradural CavVMs are discussed, encompassing malformations growing within the intradural space without direct dural involvement. In addition to classic intra-axial CavVMs, cranial nerve CavVMs, intraventricular CavVMs, and intradural extramedullary spinal CavVMs are discussed in this group, given the similar natural history and specific management challenges. Herein the authors focus on critical clinical aspects of and surgical management of these malformations based on their location and discuss optimal surgical approaches at each of these anatomical locations with illustrative cases. The commonalities of the natural history and surgical management that are dictated by anatomical considerations lend to a new location-based taxonomy for classification of CavVMs.

Positioning Transclival Tumor-Treating Fields for the Treatment of Diffuse Intrinsic Pontine Gliomas.

Diffuse intrinsic pontine glioma (DIPG) carries an extremely poor prognosis, with 2-year survival rates of <10% despite the maximal radiation therapy. DIPG cells have previously been shown to be sensitive to low-intensity electric fields in vitro. Accordingly, we sought to determine if the endoscopic endonasal (EE) implantation of an electrode array in the clivus would be feasible for the application of tumor-treating fields (TTF) in DIPG. Anatomic constraints are the main limitation in pediatric EE approaches. In our Boston Children's Hospital's DIPG cohort, we measured the average intercarotid distance (1.68 ± 0.36 cm), clival width (1.62 ± 0.19 cm), and clival length from the base of the sella (1.43 ± 0.69 cm). Using a linear regression model, we found that only clival length and sphenoid pneumatization were significantly associated with age (R2 = 0.568, p = 0.005 *; R2 = 0.605, p = 0.0002 *). Critically, neither of these parameters represent limitations to the implantation of a device within the dimensions of those currently available. Our findings confirm that the anatomy present within this age group is amenable to the placement of a 2 × 1 cm electrode array in 94% of patients examined. Our work serves to demonstrate the feasibility of implantable transclival devices for the provision of TTFs as a novel adjunctive therapy for DIPG.

Comparative Anatomic Study Between Intradural Suboccipital Retrosigmoid and Extradural Transzygomatic Middle Fossa Approaches to Expose the Trigeminal Nerve.

BACKGROUND: Meckel cave tumors are relatively rare, especially trigeminal nerve (TN) schwannomas. These tumors frequently project through the trigeminal pore, occupying the middle and posterior fossae. The most used routes to this region are the suboccipital retrosigmoid intradural approach (SORSA) and the transzygomatic middle fossa approach (TZMFA). Both approaches allow further exposure by adding intraoperative techniques, such as removing the suprameatal tubercle (retrosigmoid intradural suprameatal approach [RISA]) and the petrous apex (TZMFA-PA), respectively. This study aims to understand how TN exposure differs between both surgical approaches and how it increases by adding specific surgical maneuvers to these techniques. METHODS: Five formalin-fixed adult cadaver heads were submitted to high-resolution computed tomography and their images were loaded into the neuronavigation device. Anatomic key points were defined along the outline of the TN, and their three-dimensional spatial locations were collected following each surgical approach. This process allowed the calculation of the TN exposed area obtained through each technique. RESULTS: The mean areas of exposure of the TN were 125.9 mm2 with SORSA and 208.9 mm2 with RISA, which represents an additional mean gain of 61.92% (P = 0.047). Using TZMFA, a mean exposure of 419.24 mm2 was obtained. When TZMFA-PA was used, the mean exposed area was 486.03 mm2, representing a mean gain in the exposure area of 16.81% (P = 0.072). CONCLUSIONS: Our study suggests that TZMFA allows better exposure of TN ganglionic and postganglionic segments, and the removal of the PA adds the preganglionic segment visualization, although with less TN exposed area compared with RISA. With SORSA, the additional suprameatal tubercle removal shows the trigeminal pore and the medial margin of the central portion of the TN ganglionic segment, making it possible to expose the mouth of the Meckel cave and part of its contents.

Treating the dead; how far ought medicine go to obtain transplantable organs?

Under what circumstances, is it ethical to perform tumor surgery on a brain-dead individual? The neurosurgeons at Brigham and Women's Hospital were recently faced with such a question when asked to operate on a 28-year-old man who was pronounced brain-dead secondary to a severe brain-stem injury. His advanced directives clearly documented a desire for organ donation. During his transplant work-up, cranial imaging suggested a possible cerebellar mass of unknown etiology that was concerning for metastatic disease. Despite negative full body imaging, the neurosurgical team was asked to perform an open biopsy of the intracranial lesion to rule out occult systemic cancer. This case invites many nuanced questions related to the decisions surgeons and the broader medical community must make in the face of pursuing viable organs for the many in need. What is the moral standing and personhood eligibility of brain-dead individuals? What is the scope of medical interventions and procedures that surgeons are ethically bound to carry out? How ought the desire for increased medical intervention to try to save organs be balanced with practical limitations given limited medical resources?

Directionally encoded color track density imaging in brain tumor patients: A potential application to neuro-oncology surgical planning.

BACKGROUND: Diffusion magnetic resonance imaging white matter tractography, an increasingly popular preoperative planning modality used for pre-surgical planning in brain tumor patients, is employed with the goal of maximizing tumor resection while sparing postoperative neurological function. Clinical translation of white matter tractography has been limited by several shortcomings of standard diffusion tensor imaging (DTI), including poor modeling of fibers crossing through regions of peritumoral edema and low spatial resolution for typical clinical diffusion MRI (dMRI) sequences. Track density imaging (TDI) is a post-tractography technique that uses the number of tractography streamlines and their long-range continuity to map the white matter connections of the brain with enhanced image resolution relative to the acquired dMRI data, potentially offering improved white matter visualization in patients with brain tumors. The aim of this study was to assess the utility of TDI-based white matter maps in a neurosurgical planning context compared to the current clinical standard of DTI-based white matter maps. METHODS: Fourteen consecutive brain tumor patients from a single institution were retrospectively selected for the study. Each patient underwent 3-Tesla dMRI scanning with 30 gradient directions and a b-value of 1000 s/mm2. For each patient, two directionally encoded color (DEC) maps were produced as follows. DTI-based DEC-fractional anisotropy maps (DEC-FA) were generated on the scanner, while DEC-track density images (DEC-TDI) were generated using constrained spherical deconvolution based tractography. The potential clinical utility of each map was assessed by five practicing neurosurgeons, who rated the maps according to four clinical utility statements regarding different clinical aspects of pre-surgical planning. The neurosurgeons rated each map according to their agreement with four clinical utility statements regarding if the map 1 identified clinically relevant tracts, (2) helped establish a goal resection margin, (3) influenced a planned surgical route, and (4) was useful overall. Cumulative link mixed effect modeling and analysis of variance were performed to test the primary effect of map type (DEC-TDI vs. DEC-FA) on rater score. Pairwise comparisons using estimated marginal means were then calculated to determine the magnitude and directionality of differences in rater scores by map type. RESULTS: A majority of rater responses agreed with the four clinical utility statements, indicating that neurosurgeons found both DEC maps to be useful. Across all four investigated clinical utility statements, the DEC map type significantly influenced rater score. Rater scores were significantly higher for DEC-TDI maps compared to DEC-FA maps. The largest effect size in rater scores in favor of DEC-TDI maps was observed for clinical utility statement 2, which assessed establishing a goal resection margin. CONCLUSION: We observed a significant neurosurgeon preference for DEC-TDI maps, indicating their potential utility for neurosurgical planning.

Surgical Management in Herpes Simplex Encephalitis: Illustrative Case Report and Systematic Review of the Literature.

BACKGROUND: Herpes simplex virus (HSV) is a common cause of viral encephalitis and can result in refractory seizures. Although HSV encephalitis (HSVE) is treated primarily with acyclovir, surgery can play a role in medically intractable cases. OBJECTIVE: To systematically review cases describing surgery for the treatment of severe HSVE. We also present an illustrative case of anterior temporal lobectomy (ATL) for refractory status epilepticus in a patient with unilateral HSVE. This case demonstrates one clinical context in which surgery can be a useful adjunct. METHODS: We performed a systematic review using PubMed and Google Scholar, including case reports and series describing surgical interventions for HSVE. Clinical data were extracted from 54 publications that incorporated 67 patient cases. RESULTS: Surgical decompression occurred at a wide range of times after the onset of illness, although most patients were operated on 4 or more days after HSVE symptoms began. Numerous reports indicated that decompressive craniectomy, temporal lobectomy, and hematoma removal could treat intractably elevated intracranial pressure because of HSVE with favorable long-term outcomes. We describe an additional case in which a 52-year-old woman with HSVE developed refractory right temporal lobe seizures. After ATL, the seizures resolved with significant clinical improvement. CONCLUSION: Surgical treatment can be a useful adjunct for treatment of HSVE. There is substantial variability in the timing of surgical decompression in patients with HSVE, which can be necessary up to approximately 3 weeks after illness onset. ATL should be considered for refractory status epilepticus in HSVE with a unilateral seizure focus.

Multimodality resection of Oliveira Type IIIC* cerebellar AVM: A distinct entity.

Background: Posterior fossa AVMs constitute about 10% of AVMs and are associated with a higher rate of hemorrhage and increased morbidity and mortality rates necessitating treatment with rare exception. Cerebellar AVMs differ markedly from their supratentorial counterparts in that there are no perforating vessels involvement, drainage into the deep cerebral venous system, or presence of eloquent functional area except for the dentate nucleus. While Yasargil has classified cerebellar AVMs into seven subtypes according to their location, de Oliveira et al. have classified them using a more impactful grading system based on the size, location, and involvement of the dentate nucleus with the highest risk being III (size over 4 cm) C (mixed superficial and deep location) * (dentate involvement). In this extensive AVM with multiple arterial feeders from the SCA, AICA, and PICAs, preoperative embolization facilitates the safe surgical removal. Case Description: We present the case of resection of de Oliveira et al. IIIC* cerebellar AVM highlighting the tenets of preoperative embolization, wide surgical exposure with an extended retrosigmoid approach, arachnoidal dissection of the SAC, AICA, and PICA feeders, parenchymal dissection with preservation of the dentate nucleus, and preservation of venous drainage until complete disconnection. The patient consented to surgery after presenting with hemorrhage and developed hydrocephalus and CSF leak, managed successfully. Conclusion: de Oliveira et al. classification is highly impactful in grading posterior fossa AVMs.

Falcotentorial Meningioma Resection Through the Supracerebellar Infratentorial Approach: 2-Dimensional Operative Video.

Meningiomas are a common pineal region tumor in adults.1 They frequently reach large size with pending serious neurological consequences.1 Although they are more common in women, their presence in men might raise concerns about a higher-grade meningioma. Accordingly, their treatment starts with surgical resection. Their origin is the falcotentorial junction involving the midbrain tectum and the vital deep venous system. The torcular, transverse sinuses, cerebellar veins, straight sinus, internal occipital veins, basal veins, and internal cerebral veins are encountered requiring a cautious fine intra-arachnoidal dissection under high magnification.2 Multiple surgical approaches are described for pineal area tumors with the advantages and disadvantages of each guiding the selection of the approach.3 We believe that the main deciding factor is the relationship of the vein of Galen and its tributaries to the tumor, simply choosing the route that encounters the tumor first and the vein last. We demonstrate the surgical nuances of removing a pineal area meningioma that displaced the vein of Galen superiorly, prompting the resection through a lateral infratentorial supracerebellar approach. We currently prefer the 3/4 concord position because it provides a superb direct exposure over one cerebellar hemisphere, with the cerebellum relaxed downward while the bridging veins are not severely stretched with gravity.4 The sitting surgeon with resting arms in an ergonomic position is able to perform fine microsurgical dissection over extended time.5 The patient was a 57-year-old man with a large falcotentorial meningioma. The patient consented to the surgery and publication of his images.

Preservation of Cranial Nerves Function in Glomus Jugulare Surgery: 2-Dimensional Operative Video.

Owing to their invasive character, extreme vascularity, and critical location, glomus jugulare tumors present a formidable challenge. Techniques have been developed for safe and successful removal of even giant glomus paragangliomas.1-3 Preoperative evaluation including genetic, hormonal, and multiplicity workup4 has enhanced the safety of surgical management, as did modern preoperative embolization by eliminating excessive blood loss.5 Despite these advancements, surgical outcomes of glomus jugulare remain haunted by cranial nerve dysfunction such as facial nerve palsies and hearing loss, with lower cranial nerves dysfunction being the most morbid. These can be avoided by technical maneuvers to preserve the cranial nerves. The external ear canal is not closed to maintain conductive hearing. The facial nerve is not transpositioned and kept inside a bony protective canal. Cranial nerves IX, X, and XI are the most vulnerable because they pass through the jugular foramen ventral to the venous bulb wall. They are preserved by intrabulbar dissection that maintains a protective segment of the venous wall over the nerves. By mastering the anatomy of the upper neck, meticulous dissection is performed to preserve the course of IX, X, XI, and XII.6,7 Ligation of the jugular vein is delayed until the tumor is totally isolated to avoid diffuse bleeding.7 We present the case of a 60-yr-old woman with a glomus jugulare tumor with intradural, extradural, and cervical extension. The technical nuances of cranial nerves preservation are demonstrated. The patient consented to the procedure and publication of her images. Images at 2:12, 2:50, and 3:09 from Al-Mefty and Teixeira,6 with permission from JNSPG.

Multicentric Chordoma With Initial Resection by Bilateral Transcondylar Approach: 2-Dimensional Operative Video.

Chordoma is a rare skull base tumor with malignant behavior.1-3 It invades locally with high recurrences, metastasizes distally, and seeds after interventions.1-4 Chordoma exemplifies the malignant progression doctrine as it accumulated genetic mutations. The natural history of untreated disease is 2.4 yr on average survival.5 Best tumor control is achieved by radical resection, followed by high doses radiation. Multicentric chordoma is an ill-defined challenging entity extremely rare in the literature. However, chordoma is known for distal metastasis, particularly to the lungs, iatrogenic cerebrospinal fluid (CSF) dissemination with drop metastasis, or surgical implantation. A subset of patients present with synchronous or metachronous regional or distal neuraxial lesions associated with the initial chordoma. Patients presenting with multicentric bony axial lesions and no extra-axial metastases point toward the multicentric chordoma concept rather than local, hematogenous, or CSF spread.6-12 Biopsy of these multicentric lesions can show a spectrum of abnormalities ranging from benign notochordal tumor to chordomas confirming the multicentric hypothesis.9 We present a patient who underwent a bilateral transcondylar approach for giant craniovertebral junction chordoma and then treated with radiation and a second lesion at the C6 transverse foramen. Six years later, she presented with a chordoma at the petrous apex. The patient consented to surgery and to the publications of her image. The participants and any identifiable individuals consented to publication of his/her image. Image at 1:39 reprinted with permission from Al-Mefty O, Operative Atlas of Meningiomas. Vol 1, © LWW, 1998.

Multimodal Intraoperative Image-Driven Surgery for Skull Base Chordomas and Chondrosarcomas.

Given the difficulty and importance of achieving maximal resection in chordomas and chondrosarcomas, all available tools offered by modern neurosurgery are to be deployed for planning and resection of these complex lesions. As demonstrated by the review of our series of skull base chordoma and chondrosarcoma resections in the Advanced Multimodality Image-Guided Operating (AMIGO) suite, as well as by the recently published literature, we describe the use of advanced multimodality intraoperative imaging and neuronavigation as pivotal to successful radical resection of these skull base lesions while preventing and managing eventual complications.

Intracranial Facial Schwannomas: Multiple Distinct Entities Necessitating Tailored Management.

OBJECTIVE: Facial nerve (FN) schwannomas are extremely rare. According to their origin and involved segment(s), they constitute distinct subtypes. Intact FN function presents a management challenge, particularly in the cerebellopontine angle cisternal subtype that masquerades as a vestibular schwannoma. Fascicular-sparing technique with subtotal resection can maintain a good FN function. This study focuses on management to maintain good FN function. METHODS: A retrospective analysis of a cohort of 13 patients harboring FN schwannoma. Patient demographics, clinical findings, imaging, surgical intervention, and outcomes were analyzed. RESULTS: Five women and 8 men, with an average age of 55.3 years (39-75 years), harbored 6 cisternal, 2 ganglion, and 5 combined tumors. Average tumor size was 28.3 mm (16-50 mm). Eleven patients underwent surgery. Seven patients had fascicle-sparing technique, 5 of whom maintained their preoperative FN function, whereas 2 patients with near-total removal had a deterioration in FN function. Two patients with preoperative complete facial paralysis had gross total removal with interposition nerve graft. CONCLUSIONS: FN schwannomas management is individualized according to the subtype and the FN function at presentation. When FN function is normal, observation can be applied for prolonged period of time. At the early sign of deterioration, sub- or near-total resection with fascicle sparing technique can be performed. The cisternal subtype masquerade as vestibular schwannoma and should be recognized at the initial exposure by the appearance of finely splayed nerve fascicles at the perimetry of the tumor which elicits a motor response at low threshold stimulation.

Cerebellar High-Grade Glioma: A Translationally Oriented Review of the Literature.

World Health Organization (WHO) grade 4 gliomas of the cerebellum are rare entities whose understanding trails that of their supratentorial counterparts. Like supratentorial high-grade gliomas (sHGG), cerebellar high-grade gliomas (cHGG) preferentially affect males and prognosis is bleak; however, they are more common in a younger population. While current therapy for cerebellar and supratentorial HGG is the same, recent molecular analyses have identified features and subclasses of cerebellar tumors that may merit individualized targeting. One recent series of cHGG included the subclasses of (1) high-grade astrocytoma with piloid features (HGAP, ~31% of tumors); (2) H3K27M diffuse midline glioma (~8%); and (3) isocitrate dehydrogenase (IDH) wildtype glioblastoma (~43%). The latter had an unusually low-frequency of epidermal growth factor receptor (EGFR) and high-frequency of platelet-derived growth factor receptor alpha (PDGFRA) amplification, reflecting a different composition of methylation classes compared to supratentorial IDH-wildtype tumors. These new classifications have begun to reveal insights into the pathogenesis of HGG in the cerebellum and lead toward individualized treatment targeted toward the appropriate subclass of cHGG. Emerging therapeutic strategies include targeting the mitogen-activated protein kinases (MAPK) pathway and PDGFRA, oncolytic virotherapy, and immunotherapy. HGGs of the cerebellum exhibit biological differences compared to sHGG, and improved understanding of their molecular subclasses has the potential to advance treatment.