Nonaneurysmal perimesencephalic subarachnoid hemorrhage on noncontrast head CT: An accuracy, inter-rater, and intra-rater reliability study.

BACKGROUND AND PURPOSE: To evaluate the reliability and accuracy of nonaneurysmal perimesencephalic subarachnoid hemorrhage (NAPSAH) on Noncontrast Head CT (NCCT) between numerous raters. MATERIALS AND METHODS: 45 NCCT of adult patients with SAH who also had a catheter angiography (CA) were independently evaluated by 48 diverse raters; 45 raters performed a second assessment one month later. For each case, raters were asked: 1) whether they judged the bleeding pattern to be perimesencephalic; 2) whether there was blood anterior to brainstem; 3) complete filling of the anterior interhemispheric fissure (AIF); 4) extension to the lateral part of the sylvian fissure (LSF); 5) frank intraventricular hemorrhage; 6) whether in the hypothetical presence of a negative CT angiogram they would still recommend CA. An automatic NAPSAH diagnosis was also generated by combining responses to questions 2-5. Reliability was estimated using Gwet's AC1 (κG), and the relationship between the NCCT diagnosis of NAPSAH and the recommendation to perform CA using Cramer's V test. Multi-rater accuracy of NCCT in predicting negative CA was explored. RESULTS: Inter-rater reliability for the presence of NAPSAH was moderate (κG = 0.58; 95%CI: 0.47, 0.69), but improved to substantial when automatically generated (κG = 0.70; 95%CI: 0.59, 0.81). The most reliable criteria were the absence of AIF filling (κG = 0.79) and extension to LSF (κG = 0.79). Mean intra-rater reliability was substantial (κG = 0.65). NAPSAH weakly correlated with CA decision (V = 0.50). Mean sensitivity and specificity were 58% (95%CI: 44%, 71%) and 83 % (95%CI: 72 %, 94%), respectively. CONCLUSION: NAPSAH remains a diagnosis of exclusion. The NCCT diagnosis was moderately reliable and its impact on clinical decisions modest.

Proposed new classification for internal carotid artery segmental agenesis based on embryologic and angiographic correlation.

BACKGROUND AND PURPOSE: Internal carotid artery (ICA) agenesis is a rare anatomical variant that can involve different segments of the ICA. Although many authors proposed their own classifications of this variant basing on different criteria, none of these allows to include all the cases described in the literature. The aim of the authors is to propose a new classification that allows to include all cases of ICA agenesis described until now. MATERIALS AND METHODS: The study is based on the review of all the cases of ICA agenesis described in the literature and of the classifications already proposed. After the analysis of these cases, we looked for the limits of each classification to elaborate a new more complete one. RESULTS: We found 228 cases of ICA agenesis. Among them 40 were not includible in the existing classifications. For this reason, we proposed a new classification based on the type of flow compensation and on the embryological events that determine the different variants. The flow is compensated in Type I by the Circle of Willis; in Type II by the non-regression of an embryonic artery; in Type III by the presence of an arterio-arterial anastomosis and in Type IV by external-internal carotid arteries anastomoses. CONCLUSION: After the literature review, we proposed a new comprehensive classification based on the detailed analysis of arterial embryology. Even if all the embryological details that determine this complex variant are not completely understood yet, this classification allows to include all the cases described in literature.

Embryology of the anterior communicating artery complex: implications on possible adult variants.

The anterior cerebral artery (ACA) and the anterior communicating artery (AComA) are important arteries of the telencephalon and are prominent location for cerebral aneurysms. Their embryological development is closely linked, and explains the possible variants seen in adults. In this paper, we present details related to the development of these two arteries, focusing on some variants such as the infra-optic course of the ACA, the unpaired ACA, and the persistent primitive olfactory artery.

Immunohistochemical Markers in the Diagnosis of Calcifying Pseudoneoplasm of the Neuraxis.

BACKGROUND: Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumor-like lesion with unknown pathogenesis. It is likely under-reported due to diagnostic challenges including the nonspecific radiographic features, lack of diagnostic markers, and often asymptomatic nature of the lesions. METHODS: We performed detailed examination of 11 CAPNON specimens diagnosed by histopathology, with the help of electron microscopy and immunohistochemistry. RESULTS: Electron microscopy revealed the presence of fibrillary materials consistent with neurofilaments. In addition to some entrapped axons at the periphery of CAPNONs, we discovered that all specimens stained positive for neurofilament-light (NF-L) within the granular amorphous cores, but not neurofilament-phosphorylated (NF-p). CAPNONs also showed variable infiltration of CD8+ T-cells and a decreased ratio of CD4/CD8+ T-cells, suggesting an immune-mediated process in the pathogenesis of CAPNON. CONCLUSION: NF-L and CD4/CD8 immunostains may serve as diagnostic markers for CAPNON and shed light on its pathogenesis.

Embryology and variations of the recurrent artery of Heubner.

PURPOSE: The recurrent artery of Heubner (RAH) is a very fascinating artery for various reasons. The first one is its early development in the embryologic and phylogenic life. The second one is the discrepancy between its diameter and its functional importance and the last but not least reason is the many variations of origin and course of this artery. METHODS: For more than a century, numerous studies furnished important information about variations in origin, course, and parenchymal territory of the RAH. The most important anomaly concerning the RAH is the accessory middle cerebral artery and well-illustrates the complexity of its embryologic development. CONCLUSION: For these reasons, authors provide a review of anatomical variations of this artery that could be encountered with a particular attention given to the history and embryological knowledge.

Embryology and anatomical variations of the ophthalmic artery.

PURPOSE: The ophthalmic artery (OA) has one of the most complex anatomy and the most fascinating embryological development. METHODS: The complexity of the embryologic development of the OA resides in the implication of three different embryological systems: the carotid system, the stapedial system, and the ventral pharyngeal system. RESULTS: This explains very well the numerous variations in origin of the OA and the importance of vascular anastomoses developed with branches of the external carotid artery and with the middle meningeal artery. CONCLUSION: In this review, authors propose a comprehensive description of different hypotheses on the embryologic development of the OA and, in a second part, explain all anatomical variations and clinical implications of this artery.

Skull Base Calcifying Pseudoneoplasms of the Neuraxis: Two Case Reports and a Systematic Review of the Literature.

BACKGROUND: Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumefactive lesion. CAPNONs can mimic calcified meningiomas at the skull base. METHODS: Here, we report two cases of CAPNON and present a systematic review of the literature on skull base CAPNONs, to compare CAPNONs with calcified meningiomas. RESULTS: Case 1: A 57-year-old man presented with right-sided lower cranial neuropathies and gait ataxia. He underwent a subtotal resection of a right cerebellopontine angle lesion, with significant improvement of his gait ataxia. However, his cranial neuropathies persisted. Pathological examination of the lesion was diagnostic of CAPNON, with the entrapped nerve fibers identified at the periphery of the lesion, correlating with the patient's cranial neuropathy. Case 2: A 70-year-old man presented with progressive headache, gait difficulty, and cognitive impairment. He underwent a frontotemporal craniotomy for a near-total resection of his right basal frontal CAPNON. He remained neurologically stable 7 years after the initial resection without evidence of disease recurrence. We analyzed 24 reported CAPNONs at the skull base in our systematic review of the literature. Cranial neuropathies were present in 11 (45.8%) patients. Outcomes regarding cranial neuropathies were documented in six patients: two had sacrifice of the nerve function with surgical approaches and four had persistent cranial neuropathies. CONCLUSION: While CAPNON can radiologically and grossly mimic calcified meningiomas, they are two distinctly different pathologies. CAPNONs located at the skull base are commonly associated with cranial neuropathies, which may be difficult to reverse despite surgical intervention.

Point-of-care handheld ophthalmic ultrasound in the diagnosis and evaluation of raised intracranial pressure and Terson syndrome: a description of two cases.

BACKGROUND: Ultrasound (US) examination of the eye can be used to detect and monitor elevated intracranial pressure (ICP) and its consequences. Elevated ICP is transmitted to the contiguous optic nerve and its sheath (dura mater), thus underlying the development of papilledema and a widened sheath diameter. The US measurement of the optic nerve sheath diameter (ONSD) has previously been validated to diagnose and monitor raised ICP. The occurrence of vitreous hemorrhage in association with subarachnoid hemorrhage (SAH)-i.e., Terson syndrome-can also be easily diagnosed using ophthalmic US. Because of its relevance in anesthesia and critical care, we describe how to perform the technique illustrated by two cases. CASE PRESENTATIONS: A 72-yr-old man with hydrocephalus secondary to a SAH developed raised ICP following the removal of an external ventricular drainage (EVD) system. Daily ONSD measurements using handheld US allowed us to diagnose and monitor the progression and resolution of the intracranial hypertension following the placement of a second EVD system. We also describe the steps used to obtain ONSD measurements during the ophthalmic US examination of a 53-yr-old woman who presented with a stage IV SAH with concomitant bilateral vitreous hemorrhages or Terson syndrome. CONCLUSION: Ophthalmic US using a handheld device to measure and monitor ONSD at the bedside is useful in diagnosing and monitoring the progression of intracranial hypertension following EVD removal in a patient with hydrocephalus secondary to SAH. Ophthalmic US can also be used to identify concomitant vitreous hemorrhage that is associated with a worse prognosis.

How I do it: lateral supra-cerebellar infra-tentorial approach for P2-P3 junction cerebral aneurysms.

BACKGROUND: P2-P3 junction aneurysms are challenging to treat surgically because of their frequent complex morphology and their location deep in close proximity to the midbrain. The sub-temporal route requires significant retraction of the temporal lobe in addition to potential injury to the vein of Labbe. We describe the technique for treating such aneurysms via a lateral supra-cerebellar infra-tentorial (LSCIT) approach, which eliminates manipulation of the temporal lobe. METHOD: Cadaveric dissection provided comprehensive understanding of relevant anatomy. Intraoperative video shows clipping of the aneurysm using a LSCIT approach. CONCLUSION: LSCIT approach allows safe clipping of P2-P3 aneurysms with minimal brain manipulation.

Arteriovenous malformations of the posterior fossa: a systematic review.

BACKGROUND: Posterior fossa arteriovenous malformations (pfAVMs) can be challenging lesions to manage. AVMs in this location may have distinct features compared with supratentorial AVMs. Our aim was to systematically review the literature on the presenting characteristics of pfAVMs and compare clinical and angiographic outcomes after the various types of treatment employed. METHODS: The review was conducted according to the Cochrane Collaboration guidelines. Electronic databases from 1900 to March 2018 were searched and complemented by hand-searching and cross-referencing. Articles were categorized into (i) AVM studies that included those in the posterior fossa, (ii) those that focused exclusively on pfAVM, and (iii) those that further specified a cerebellar or brainstem location of the AVM. RESULTS: Seventy-seven articles with 4512 pfAVM patients were retained for analyses. Compared with historical supratentorial controls, pfAVMs were reported to more frequently present with rupture, to more commonly have associated arterial aneurysms, and to more frequently lead to poor clinical and angiographic outcomes. The quality of the literature and lack of standardization of outcome reporting precluded performing a meta-analysis on the results of the various different treatment modalities. CONCLUSIONS: Posterior fossa AVMs may have some distinct features compared with supratentorial AVMs. The available reports on pfAVMs are not sufficiently standardized to provide reliable guidance for patient management decisions. This goal will require future studies to be multicentric and to focus on standardized, repeatable clinical and angiographic outcomes.

Innate immunity activation in the early brain injury period following subarachnoid hemorrhage.

BACKGROUND: Aneurysmal subarachnoid hemorrhage (SAH) is a catastrophic disease with devastating consequences, including a high mortality rate and severe disabilities among survivors. Inflammation is induced following SAH, but the exact role and phenotype of innate immune cells remain poorly characterized. We investigated the inflammatory components of the early brain injury in an animal model and in SAH patients. METHOD: SAH was induced through injection of blood in the subarachnoid space of C57Bl/6 J wild-type mice. Prospective blood collections were obtained at 12 h, days 1, 2, and 7 to evaluate the systemic inflammatory consequences of SAH by flow cytometry and enzyme-linked immunosorbent-assay (ELISA). Brains were collected, enzymatically digested, or fixed to characterize infiltrating inflammatory cells and neuronal death using flow cytometry and immunofluorescence. Phenotypic evaluation was performed at day 7 using the holding time and footprint tests. We then compared the identified inflammatory proteins to the profiles obtained from the plasma of 13 human SAH patients. RESULTS: Following SAH, systemic IL-6 levels increased rapidly, whereas IL-10 levels were reduced. Neutrophils were increased both in the brain and in the blood reflecting local and peripheral inflammation following SAH. More intracerebral pro-inflammatory monocytes were found at early time points. Astrocyte and microglia activation were also increased, and mice had severe motor deficits, which were associated with an increase in the percentage of caspase-3-positive apoptotic neurons. Similarly, we found that IL-6 levels in patients were rapidly increased following SAH. ICAM-1, bFGF, IL-7, IL-12p40, and MCP-4 variations over time were different between SAH patients with good versus bad outcomes. Moreover, high levels of Flt-1 and VEGF at admission were associated with worse outcomes. CONCLUSION: SAH induces an early intracerebral infiltration and peripheral activation of innate immune cells. Furthermore, microglia and astrocytic activation are present at later time points. Our human and mouse data illustrate that SAH is a systemic inflammatory disease and that immune cells represent potential therapeutic targets to help this population of patients in need of new treatments.

Clipping of a PICA aneurysm located on the contralateral side of its parent vertebral artery in front of the brainstem: how I do it.

BACKGROUND: Vertebro-PICA aneurysms may be challenging because of their relationship with the brainstem and the lower cranial nerves, especially when the vertebral artery is tortuous and the aneurysm is located in front of the brainstem, contralaterally to the parent vertebral artery. We describe the surgical technique for safe approach. METHOD: Cadaveric dissection performed by the authors, provided comprehensive understanding of relevant anatomy. Intraoperative photos and videos show clipping of the aneurysm using a combined midline and far-lateral suboccipital craniotomy with a para-condylar extension. The literature reviews potential complications. CONCLUSION: This combined approach allows safe clipping of such PICA aneurysms.

Surgical management of giant sphenoid wing meningiomas encasing major cerebral arteries.

OBJECTIVE Sphenoid wing meningiomas are a heterogeneous group of tumors with variable surgical risks and prognosis. Those that have grown to a very large size, encasing the major cerebral arteries, are associated with a high risk of stroke. In reviewing the authors' series of giant sphenoid wing meningiomas, the goal was to evaluate how the extent of the tumor's invasion of surrounding structures affected the ability to safely remove the tumor and restore function. METHODS The authors conducted a retrospective study of a series of giant sphenoid wing meningiomas operated on between 1996 and 2016. Inclusion criteria were meningiomas with a globoid component ≥ 6 cm, encasing at least 1 major intradural cerebral artery. Extent of resection was measured according to Simpson grade. RESULTS This series included 12 patients, with a mean age of 59 years. Visual symptoms were the most common clinical presentation. There was complete or partial encasement of all 3 major cerebral arteries except for 3 cases in which only the anterior cerebral artery was not involved. The lateral wall of the cavernous sinus was invaded in 8 cases (67%) and the optic canal in 6 (50%). Complete resection was achieved in 2 cases (Simpson grades 2 and 3). In the remaining 10 cases of partial resection (Simpson grade 4), radical removal (> 90%) was achieved in 7 cases (70%). In the immediate postoperative period, there were no deaths. Four of 9 patients with visual deficits improved, while the 5 others remained unchanged. Two patients experienced transient neurological deficits. Other than an asymptomatic lacuna of the internal capsule, there were no ischemic lesions following surgery. Tumor recurrence occurred in 5 patients, between 24 and 168 months (mean 61 months) following surgery. CONCLUSIONS Although these giant lesions encasing major cerebral arteries are particularly treacherous for surgery, this series demonstrates that it is possible to safely achieve radical removal and at times even gross-total resection. However, the risk of recurrence remains high and larger studies are needed to see if and how improvement can be achieved, whether in surgical technique or technological advances, and by determining the timing and modality of adjuvant radiation therapy.

Surgical clipping or endovascular coiling for unruptured intracranial aneurysms: a pragmatic randomised trial.

BACKGROUND: Unruptured intracranial aneurysms (UIAs) are increasingly diagnosed and are commonly treated using endovascular treatment or microsurgical clipping. The safety and efficacy of treatments have not been compared in a randomised trial. How to treat patients with UIAs suitable for both options remains unknown. METHODS: We randomly allocated clipping or coiling to patients with one or more 3-25 mm UIAs judged treatable both ways. The primary outcome was treatment failure, defined as: initial failure of aneurysm treatment, intracranial haemorrhage or residual aneurysm on 1-year imaging. Secondary outcomes included neurological deficits following treatment, hospitalisation >5 days, overall morbidity and mortality and angiographic results at 1 year. RESULTS: The trial was designed to include 260 patients. An analysis was performed for slow accrual: 136 patients were enrolled from 2010 through 2016 and 134 patients were treated. The 1-year primary outcome, available for 104 patients, was reached in 5/48 (10.4% (4.5%-22.2%)) patients allocated surgical clipping, and 10/56 (17.9% (10.0%-29.8%)) patients allocated endovascular coiling (OR: 0.54 (0.13-1.90), p=0.40). Morbidity and mortality (modified Rankin Scale>2) at 1 year occurred in 2/48 (4.2% (1.2%-14.0%)) and 2/56 (3.6% (1.0%-12.1%)) patients allocated clipping and coiling, respectively. New neurological deficits (15/65 vs 6/69; OR: 3.12 (1.05-10.57), p=0.031), and hospitalisations beyond 5 days (30/65 vs 6/69; OR: 8.85 (3.22-28.59), p=0.0001) were more frequent after clipping. CONCLUSION: Surgical clipping or endovascular coiling of UIAs did not show differences in morbidity at 1 year. Trial continuation and additional randomised evidence will be necessary to establish the supposed superior efficacy of clipping.

Minimally invasive costotransversectomy for the resection of large thoracic dumbbell tumors.

BACKGROUND: Due to their important size and complex localization, the management of thoracic dumbbell tumors is challenging, frequently requiring the need for an anterior approach. Our study aims to first report the feasibility and safety of a single-stage posterior minimally invasive procedure in achieving complete resection of voluminous thoracic dumbbell tumors. METHODS: We retrospectively reviewed the medical records of five consecutive patients, who underwent the minimally invasive resection of a type III thoracic dumbbell tumor in our institution between March 2007 and March 2012. There were two men and three women, with a mean age at diagnosis of 57 years (range 41-68 years). After the placement of a non-expandable tubular retractor under fluoroscopic control, a costotransversectomy was achieved. By moving the retractor in all directions, the tumor was largely exposed and resected with the cavitron ultrasonic surgical aspirator. Clinical and radiological monitoring was performed before discharge, at 6 months, 1 year and 2 years. RESULTS: No major intraoperative complication was reported. Gross total resection was achieved in four patients. The mean operative time was 219 mins (range 75-540 mins) and the mean estimated blood loss was 230 ml (range 50-500 ml). No postoperative complication was reported. The mean length of hospital stay was 3.6 days (range 2-6 days) and all patients were discharged home. Histological analysis confirmed the diagnosis of grade 1 schwannoma in four patients and revealed a hemangiopericytoma in one patient. No tumor recurrence was noted with a mean follow up period of 46 months (range 32-54 months). CONCLUSION: Thoracic dumbbell tumors can be safely and completely resected using a single-stage minimally invasive procedure. The costotransversectomy can be performed through a non-expandable retractor allowing sufficient access to all parts of the tumor.

The Treatment of Brain Arteriovenous Malformation Study (TOBAS): A preliminary inter- and intra-rater agreement study on patient management.

OBJECT: The best management of brain arteriovenous malformation (bAVM) patients remains unknown. Randomized allocation may be more readily accepted when there is uncertainty and disagreement regarding the management of potential participants. In planning for a trial, we aimed to assess variability and agreement among physicians managing bAVM patients. METHODS: A portfolio composed of 35 patients was sent to 47 clinicians of various specialties managing bAVM patients. For each patient, physicians were asked their best management decision (surgery/embolization/radiosurgery/conservative), their confidence level, and whether they would include the patient in a randomized trial comparing conservative and curative management. Seven physicians, who had access to all images of each patient, independently responded twice, to assess inter and intra-rater agreement using kappa statistics. RESULTS: The inter-rater agreement (30 raters, including 16 neuroradiologists) for best management decision was only "fair" (κ [95%CI]=0.210[0.157; 0.295]). Agreement remained below 'substantial' (κ<.6) between physicians of the same specialty, and when no distinctions were made between various treatments (when responses were dichotomized as conservative versus curative). With access to all images the inter-rater agreement remained fair. The intra-rater agreement reached "substantial" only for the dichotomized decisions. Responding clinicians were willing to include 54.4% of patients (mainly unruptured bAVMs) in a randomized trial. CONCLUSION: There is a lack of agreement among clinicians involved in the management of bAVM patients. In this study a substantial proportion of clinicians were willing to offer randomized allocation of management options to a substantial number of patients.

Supracerebellar transtentorial removal of a large tentorial tumor.

Large tumors located on the upper surface of the tentorium, extending to the posterior edge of the tentorial incisura and affecting the posterior temporo-mesial region, are challenging to reach without damaging the surrounding brain. Typically, these lesions are approached through a subtemporal or a transcortical transtemporal corridor. To avoid temporal lobe transgression or retraction, and venous drainage compromise, we use a supracerebellar transtentorial (SCTT) approach which gives a direct exposure to the posterior temporo-medial region and the posterior incisural space. In this video, we demonstrate the surgical technique of the SCTT approach. The video can be found here: https://youtu.be/g3ilMO8vo4g .

Using the trans-lamina terminalis route via a pterional approach to resect a retrochiasmatic craniopharyngioma involving the third ventricle.

Retrochiasmatic craniopharyngiomas involving the anterior third ventricle are challenging to access. Although the pterional approach is a common route for suprasellar lesions, when the craniopharyngioma extends behind the chiasma into the third ventricle, access is even more difficult, and the lamina terminalis may offer a good working window. The translamina terminalis approach provides direct access to the retrochiasmatic portion of the tumor with minimal brain retraction and no manipulation of the visual nerves. In this video, we emphasize the utility of using the lamina terminalis corridor to resect the retrochiasmatic intraventricular portion of a craniopharyngioma. The video can be found here: https://youtu.be/hrLNC0hDKe4 .