A review of histopathologic and radiologic features of non-atherosclerotic pathologies of the extracranial carotid arteries.

Diseases of the carotid arteries can be classified into different categories based on their origin. Atherosclerotic carotid disease remains the most encountered arterial wall pathology. However, other less-common non-atherosclerotic diseases can have detrimental clinical consequences if not appropriately recognized. The underlying histological features of each disease process may result in imaging findings that possess features that are obvious of the disease. However, some carotid disease processes may have histological characteristics that manifest as non-specific radiologic findings. The purpose of this manuscript is to review various non-atherosclerotic causes of carotid artery disease as well as their histologic-radiologic characteristics to aid in the appropriate recognition of these less-commonly encountered pathologies.

Carotid Artery Tortuosity and Internal Carotid Artery Plaque Composition.

BACKGROUND: Little is known about the association between carotid artery tortuosity and internal carotid artery atherosclerosis. This study sought to evaluate the associations between various types of arterial tortuosity and vulnerable plaque components on magnetic resonance angiography (MRA). MATERIAL AND METHODS: A retrospective review was completed of 102 patients who had undergone MRA neck imaging, with intraplaque hemorrhage (IPH) present in one or both cervical internal carotid arteries (ICA). Each ICA was assessed for two categories of tortuosity: variant arterial pathway(s) (retrojugular and/or retropharyngeal) and abnormal curvature (kinks, loops, and/or coils). All ICA plaques were assessed for the presence or absence of intraplaque hemorrhage (IPH), lipid-rich necrotic core (LRNC), ulceration, and enhancement, as well as the volume of IPH and degree of luminal stenosis. RESULTS: The mean age of included patients was 73.5 years (SD = 9.0 years) and 88 (86.3%) subjects were male. The left carotid plaque was significantly more likely to have IPH (68.6% vs. 47.1%; p = 0.02). The left ICA was more likely to have a retrojugular course (22% vs. 9.9%; p = 0.002) and any variant arterial course (26.5% versus 14.67%, p = 0.01). On the right there was an association between the presence of a LRNC and retropharyngeal and/or retrojugular arterial pathway (p = 0.03). On the left there was an association between the presence of any abnormal arterial curvature and IPH volume (p = 0.03). Neither association met the adjusted statistical threshold after Bonferroni correction, with alpha set at 0.0028. CONCLUSION: ICA tortuosity is not associated with carotid artery plaque composition, and likely does not play a role in the development of high-risk plaques.

Intraplaque hemorrhage on magnetic resonance angiography: How often do signal abnormalities persist on follow-up imaging?

BACKGROUND AND PURPOSE: Intraplaque hemorrhage (IPH) in carotid atherosclerosis demonstrates increased signal on magnetic resonance angiography images. Little remains known about how this signal changes on subsequent examinations. MATERIALS AND METHODS: A retrospective observational study was completed of patients that had IPH on a neck MRA between 1/1/2016 and 3/25/2021, defined as ≥ 200 % signal intensity of the sternocleidomastoid muscle on MPRAGE images. Examinations were excluded if the patients had undergone carotid endarterectomy between examinations or had poor quality imaging. IPH volumes were calculated by manually outlining IPH components. Up to 2 subsequent MRAs, if available, were assessed for both the presence and volume of IPH. RESULTS: 102 patients were included, of which 90 (86.5 %) were male. IPH was on the right in 48 patients (average volume = 174.0 mm3), and on the left in 70 patients (average volume 186.9 mm3). 22 had at least one follow-up (average 444.7 days between exams), and 6 had two follow-up MRAs (average 489.5 days between exams). On the first follow-up, 19 (86.4 %) plaques had persistent hyperintense signal in the region of IPH. The second follow-up showed persistent signal in 5/6 plaques (88.3 %). Combined volume of IPH from right and left carotid arteries did not significantly decrease on the first follow-up exam (p = 0.08). CONCLUSIONS: IPH usually retains hyperintense signal on follow-up MRAs, possibly representing recurrent hemorrhage or degraded blood products.

Radiculomedullary arterial contributions to the thoracolumbar spinal cord: an angiographic study.

BACKGROUND: Anterior (ASA) and posterior spinal arteries (PSA) receive vital collateral flow from radiculomedullary (RM) arteries. The purpose of this study was to review a series of spinal angiograms in order to characterize normal RM arterial anatomy. METHODS: The reports of digital subtraction spinal angiograms from consecutive patients from our institution from 2002-2019 were retrospectively reviewed. The RM contributions to both the ASA and PSA were characterized by noting laterality, spinal level and multiplicity. RESULTS: Overall, 336 spinal angiograms from 336 patients were included. Regarding RM input to the ASA, 328 patients (97.6%) had at least one RM contribution to the ASA. Forty-six patients (46/328, 14.1%) had multiple RM ASA contributions. Three hundred and eighty-one total RM with input to the ASA were visualized. Ninety-five RM of the ASA (95/381, 24.9%) were located on the right, 286 (75.1%) on the left. Three hundred and twenty-four RM arteries (85.0%) arose between T8 and L2: 246 (64.5% overall) were located on the left, and 78 (20.5%) on the right. Sixty-one patients (18.2%) had at least one visualized RM contribution to the PSA: 16 patients (16/61, 26.2%) had more than one RM contribution to the PSA. Eighty-seven total RM contributions to the PSA were visualized. Eighty-one (93.1%) RM arose between T6 and L1, 52 of which (59.8% overall) were from the left, and 29 (33.3%) from the right. CONCLUSIONS: RM anastomoses with both the ASA and PSA most-commonly originate from the left-sided T6-L2 spinal levels. Multiple RM contributions to the ASA or PSA are less common.

Vessel wall imaging features of Moyamoya disease in a North American population: patterns of negative remodelling, contrast enhancement, wall thickening, and stenosis.

BACKGROUND: This study characterized vessel wall imaging (VWI) features of Moyamoya disease (MMD) in a predominantly adult population at a North American center. METHODS: Consecutive patients with VWI were included. Twelve arterial segments were analyzed for wall thickening, degree and pattern of contrast enhancement, and remodeling. RESULTS: Overall, 286 segments were evaluated in 24 patients (mean age = 36.0 years [range = 1-58]). Of 172 affected segments, 163 (95%) demonstrated negative remodeling. Complete vessel wall obliteration was most frequent in the proximal M1 (17/48, 35%). Affected segments enhanced in 72/172 (42%) (n = 15 for grade II; n = 54 for concentric and n = 18 for eccentric); 20 of 24 (83%) patients had at least one enhancing segment. Both enhancing and non-enhancing segments were present in 19/20 (95%) patients. Vessel wall enhancement was most common in the proximal segments and correlated to the degree of stenosis (p < 0.001), and outer wall diameter (p < 0.001), but not disease duration (p = 0.922) or Suzuki score (p = 0.477). Wall thickening was present in 82/172 (48%) affected segments and was associated with contrast enhancement (p < 0.001), degree of stenosis (p < 0.001), and smaller outer wall diameter (p = 0.004). CONCLUSION: This study presents VWI findings in North American patients with MMD. Negative remodeling was the most common finding. Most patients had both enhancing and non-enhancing abnormal segments. Vessel wall enhancement was most common in proximal segments, variable in pattern or degree and was correlated to the degree of stenosis and smaller outer wall diameter.

Biology and Hemodynamics of Aneurysm Rupture.

Predicting rupture risk in intracranial aneurysms is among one of the most critical questions in vascular surgery. The processes that govern an aneurysm growth are multifaceted and complex, but may be summarized into three components: hemodynamics, biology, and mechanics. We review and connect the literature in the three disciplines, identifying considerable strides in recent history and current gaps in research. Taken together, the findings from each field elucidate how and why certain aneurysms rupture, whereas others remain stable. These parameters could eventually inform a translatable predictive model that optimizes risk evaluation and physician's decision-making in treatment options for aneurysms.

Comparison of Stroke Risk Factors between Symptomatic and Asymptomatic Patients in a North American Moyamoya Disease Cohort.

BACKGROUND: Risk factors for stroke in symptomatic and asymptomatic moyamoya disease (MMD) patients have recently been reported in a Japanese cohort. Such information in a North American population is lacking. OBJECTIVE: We sought to elucidate the prevalence of stroke risk factors among North American patients with ischemic, hemorrhagic, and asymptomatic MMD. METHODS: We retrospectively reviewed our institution's database between 1990 and 2021. We excluded cases of moyamoya syndrome. We divided 119 patients into 3 groups based on the onset pattern; ischemic, hemorrhagic, and asymptomatic. We compared the prevalence of well-known stroke risk factors (diabetes, hypertension, etc.) between these three groups of patients. In the asymptomatic group, we analyzed the prevalence of cerebrovascular events on follow-up from the time of diagnosis. RESULTS: Overall, 119 patients with MMD were available with predominately White ethnicity (80.7%). The mean age was 39 years, and 73.9% were female. Patients presented with ischemic stroke (82%) and hemorrhagic stroke (11%); 7% of patients were asymptomatic. The prevalence of stroke risk factors did not differ among ischemic, hemorrhagic, or asymptomatic MMD patients. In 8 asymptomatic patients, there was 81.8 months (SD ±51.0) of follow-up, and none of them developed any cerebrovascular events. CONCLUSIONS: No significant differences in the prevalence of stroke risk factors between MMD cohorts were found, corroborating evidence provided in a recent Japanese-based study. There were no apparent associations between stroke risk factors and interval cerebrovascular events in an asymptomatic group of MMD patients.

Carotid artery endarterectomy in patients with symptomatic non-stenotic carotid artery disease.

OBJECT: We sought to determine the safety and efficacy in secondary stroke prevention of carotid endarterectomy (CEA) in patients with symptomatic non-stenotic carotid artery disease (SyNC). METHODS: This was a single-centre retrospective case series. All patients who underwent CEA for unilateral anterior circulation cerebrovascular events with ipsilateral <50% carotid stenosis from 2002 to 2020 were included. Imaging hallmarks including the degree of luminal stenosis and the presence of various vulnerable plaque characteristics (eg, intraplaque haemorrhage (IPH) on MR angiography, ulceration or low-density plaque on CT angiography) were assessed. The presence of vulnerable plaque characteristics was compared between arteries ipsilateral to the ischaemic event and contralateral arteries. The prevalence of perioperative/intraoperative complications, as well as recurrent ischaemic events at follow-up was determined. RESULTS: Thirty-two patients were included in the analysis, of which 25.0% were female. Carotid arteries ipsilateral to an ischaemic event had a significantly higher prevalence of IPH when compared with contralateral arteries (80.0% vs 0.0%; p<0.001). There were no intraoperative complications. One patient (3.1%) developed symptoms of transient ipsilateral ischaemia 1 day following CEA which resolved without treatment. In a median follow-up of 18.0 months (IQR 5.0-36.0), only one patient (3.1%) experienced a transient neurologic deficit with complete resolution (annualised rate of recurrent stroke after CEA of 1.5% for a total follow-up of 788 patient-months following CEA). All other patients (31/32, 96.9%) were free of recurrent ischaemic events. CONCLUSION: CEA appears to be safe and well-tolerated in patients with SyNC. Additional studies with larger cohorts and longer follow-up intervals are needed in order to determine the role of CEA in this patient population.

Normalized intraplaque hemorrhage signal on MP-RAGE as a marker for acute ischemic neurological events.

PURPOSE: This study sought to validate whether the signal intensity ratio (SIR) of carotid intraplaque hemorrhage (IPH) was associated with acute ischemic neurologic events. METHODS: A retrospective review was completed of consecutive patients that underwent neck magnetic resonance angiography using magnetization prepared-rapid gradient echo (MP-RAGE) and T1-CUBE sequences between 2017 and 2020. Patients with magnetic resonance evidence of IPH were included. SIRs were measured by comparing the maximum IPH signal with the mean intramuscular signal from the adjacent sternocleidomastoid. Patients were stratified into ischemic or non-ischemic groups based on the presence of acute ipsilateral ischemic events (stroke, retinal artery occlusion). Logistic regression analysis was performed to determine if increasing IPH SIR was associated with an increased risk of ipsilateral ischemic events. RESULTS: Of 85 included patients (85 arteries), 66 were male (77.6%). Mean age was 71.0 (SD ± 11.1). There were 70 arteries with IPH that were ipsilateral to an ischemic event, and 15 that belonged to patients without an ischemic event. No association was found between increasing IPH SIR seen on MP-RAGE (odds ratio (OR): 0.82; 95% confidence interval (CI): 0.58-1.4; P = 0.43) or T1-CUBE sequences (OR: 0.85; 95% CI: 0.53-1.5; P = 0.56). CONCLUSIONS: There was no association between the SIR of IPH and acute ischemia on either MP-RAGE or T1-CUBE sequences. Further investigation is required prior to widespread acceptance of SIR as a predictive imaging marker of symptomatic carotid plaque.

The cerebrofacial metameric syndromes: An embryological review and proposal of a novel classification scheme.

The cerebrofacial metameric syndromes are a group of congenital syndromes that result in vascular malformations throughout specific anatomical distributions of the brain, cranium and face. Multiple reports of patients with high-flow or low-flow vascular malformations following a metameric distribution have supported this idea. There has been much advancement in understanding of segmental organization and cell migration since the concept of metameric vascular syndromes was first proposed. We aim to give an updated review of these embryological considerations and then propose a more detailed classification system for these syndromes, predominately incorporating the contribution of neural crest cells and somitomeres to the pharyngeal arches.

Imaging of benign cervicofacial vascular anomalies and associated syndromes.

Cervicofacial vascular anomalies can result in morbidity, pain, and cosmetic concerns in affected individuals. Each anomaly has its own unique natural history, treatment, and associations with underlying genetic syndromes. For optimal patient care, it is important for the neuroradiologist to accurately recognize and characterize these entities to ensure appropriate treatment and management. In this review, we discuss the general characteristics, classifications, and imaging features associated with the most common vascular anomalies such as hemangiomas, arteriovenous malformations and fistulas, capillary malformations, venous malformations, and lymphatic malformations in the context of associated syndromes. Additionally, we discuss novel imaging techniques that aid in identifying these vascular anomalies.

Endarterectomy for symptomatic non-stenotic carotids: a systematic review and descriptive analysis.

OBJECTIVE: To systematically analyse prior reports of carotid endarterectomy (CEA) performed in cases of ≤50% carotid stenosis in order to understand patient tolerance and potential benefit. METHODS: A systematic review and descriptive analysis was performed in concordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. An English-language search was performed of online databases using librarian-selected search terms. Abstracts were reviewed for relevance which included mention of carotid endarterectomy and stenosis. Prospective or retrospective observational cohort studies that reported series of patients who underwent endarterectomy for minimal (≤50%) luminal stenosis with reported outcomes were included. RESULTS: Six studies (which included our institutional series) with a total of 143 patients met the inclusion criteria. The weighted mean age at the time of CEA was 72.3 years; 113 (79.0%) were male. 55.8% of patients with available data had recurrent ipsilateral ischaemic events despite medical therapy. Two patients out of 129 with available perioperative data (1.6%) had perioperative MRI findings of acute ischaemic stroke, both within the hemisphere contralateral to the side of CEA. Of the 138 patients with available follow-up (mean, 36 months), none had recurrent ipsilateral ischaemic events. CONCLUSIONS: Endarterectomy for symptomatic carotid disease causing ≤50% stenosis may be a potentially beneficial strategy to prevent stroke recurrence. Studies with robust methodology are needed to draw more definitive conclusions in terms of the safety and efficacy of endarterectomy for minimal stenosis with vulnerable features relative to intensive medical therapy.

Carotid Plaques From Symptomatic Patients With Mild Stenosis Is Associated With Intraplaque Hemorrhage.

Carotid plaque vulnerability features beyond the degree of stenosis may play a key role in the pathogenesis and recurrence of ischemic cerebrovascular events. This study sought to compare intraplaque hemorrhage (IPH) as a marker of plaque vulnerability in symptomatic patients with mild (<50%), moderate (50%-69%), and severe (≥70%) carotid artery stenosis. We included patients who experienced ischemic cerebrovascular events with no other identifiable sources and underwent carotid endarterectomy for mild (n=32), moderate (n=47), and severe (n=58) carotid artery stenosis. The degree of stenosis and imaging hallmarks were assessed by computed tomography angiography or magnetic resonance angiography. Plaque specimens were stained with hematoxylin and eosin and Movat pentachrome staining. Carotid plaques of patients with mild stenosis had a higher extent of IPH (%) on tissue analysis compared with patients with moderate (mild, 15.7% [interquartile range, 7.8%-26.7%]; moderate, 3.9% [0.0%-9.2%]; P<0.001) and severe carotid artery stenosis (mild, 15.7% [interquartile range, 7.8%-26.7%]; severe, 2.5% [interquartile range, 0.0%-11.2%]; P<0.001). When considering the degree of carotid artery stenosis as a continuous variable, a lower lumen narrowing was associated with higher extent of IPH (P<0.001; R, -0.329). Our major finding is the association of IPH with mild carotid artery stenosis based on histological analysis. The current study may suggest that IPH potentially plays a role in the mechanism of stroke in patients with nonobstructive carotid stenosis.

The foramen ovale "mirage" and how this impacts percutaneous cannulation for treatment of trigeminal neuralgia: a report of two cases.

Percutaneous stereotactic radiofrequency rhizotomy (PSR) for trigeminal neuralgia most commonly utilizes 2D fluoroscopy for intraoperative needle guidance into the foramen ovale (FO). We describe two cases in which needle advancement into FO was unachievable despite appropriate needle placement on biplane fluoroscopy. Intraoperative multiplanar reconstruction was helpful in more accurately depicting foraminal anatomy which allowed the manipulation of the tip of the needle, which was followed by successful FO cannulation. We propose that this "mirage" is likely created by the inherent nature of X-ray-based fluoroscopy in which the FO appears to be readily penetrable, when in fact the 3D anatomy actually prevents cannulation.

Minimally-invasive tubular retraction ports for intracranial neurosurgery: History and future perspectives.

Brain retraction is a necessary yet potentially damaging requirement of accessing lesions located in deep structures. The development of minimally-invasive tubular retractors (MITRs) provides the theoretical advantage of maximizing visualization of and access to deep-seated lesions, all while minimizing collateral tissue damage. These advantages make MITRs preferable to traditional bladed retractors in the majority of deep-seated lesions. Several commercially-available MITR systems currently exist and have been shown to aid in achieving excellent outcomes with acceptable safety profiles. Nevertheless, important drawbacks to currently-available MITR systems exist. Continued pursuit of an ideal MITR system that provides maximal visualization and access to deep-seated lesions while minimizing retraction-related tissue damage is therefore important. In this review, we discuss the historical development of MITRs, the advantages of MITRs compared to traditional bladed retractors, and opportunities to improve the development of prospective MITRs.

Implementation and Rationale for a Unified Clinical and Imaging Protocol for Evaluation and Treatment of Moyamoya Angiopathy: A Single Institutional Experience.

Moyamoya disease (MMD) is a complex and incompletely-understood cerebrovascular pathological entity that requires thorough clinical and imaging evaluation. Moyamoya is rare, thereby making the establishment of an effective, thorough and interdisciplinary patient evaluation protocol challenging, even within specialized referral centers. Nevertheless, implementation of such a protocol is crucial in order to provide the best possible evaluation and treatment for MMD patients. Here, we describe our institution's implementation of, rationale for, and experience with a comprehensive multidisciplinary collaboration and evaluation strategy for adult patients with moyamoya. This evaluation course consists of, first of all, a thorough clinical and laboratory evaluation with a vascular neurologist. This is followed by a comprehensive imaging assessment which evaluates angiographic and parenchymal features, in addition to cerebrovascular functionality. Finally, appropriate referrals are made to consulting services as indicated, which includes vascular neurosurgery. These steps are described in detail herein.

Subarachnoid Hemorrhage of Unknown Cause: Distribution and Role of Imaging.

Subarachnoid hemorrhage of unknown cause represents approximately 10% to 15% of nontraumatic subarachnoid hemorrhages. The key factors in determining the management strategy for a presumed nonaneurysmal subarachnoid hemorrhage are the distribution, location, and amount of subarachnoid blood. Hemorrhage distribution on computed tomography can be categorized as follows: perimesencephalic, diffuse, sulcal, and primary intraventricular. The extent of the workup required in determining the cause of hemorrhage depends on the distribution of blood. The authors review the potential causes, differential diagnoses, and acute and long-term follow-up strategies in patients with subarachnoid hemorrhage of unknown cause.