Bilateral internal carotid artery hypoplasia presenting with watershed territory infarcts.

Internal carotid artery hypoplasia is a rare vascular anomaly that can lead to various neurological symptoms due to altered cerebral blood flow. We present a case of a 36 years old female who presented to us with forgetfulness and right sided weakness. She was ultimately diagnosed with bilateral internal carotid artery hypoplasia through imaging studies. This case highlights the importance of considering vascular anomalies in patients presenting with neurological symptoms and the significance of comprehensive diagnostic evaluation for appropriate management.

Valoctocogene roxaparvovec gene therapy provides durable haemostatic control for up to 7 years for haemophilia A.

INTRODUCTION: Valoctocogene roxaparvovec is an adeno-associated virus vector serotype 5 (AAV5)-mediated gene therapy approved for severe haemophilia A (HA). AIM: To report the safety and efficacy of valoctocogene roxaparvovec 7 years after dosing in a phase 1/2 clinical study (NCT02576795). METHODS: Males ≥18 years with severe HA (factor VIII [FVIII] ≤1 international unit [IU]/dL) who were previously receiving exogenous FVIII and had no history of FVIII inhibitors or anti-AAV5 antibodies received valoctocogene roxaparvovec treatment and were followed for 7 (6 × 1013 vg/kg; n = 7) and 6 (4 × 1013 vg/kg; n = 6) years. RESULTS: In the last year, one participant in each cohort reported treatment-related adverse events (AEs): grade 1 (G1) hepatomegaly (6 × 1013), and G1 splenomegaly and G1 hepatic steatosis (4 × 1013). During all follow-up, mean annualized treated bleeds and exogenous FVIII infusion rates were ≥88% lower than baseline values. At years 7 and 6, mean (median) FVIII activity (chromogenic assay) was 16.2 (10.3) and 6.7 (7.2) IU/dL in the 6 × 1013 (n = 5) and 4 × 1013 (n = 4) cohorts, respectively, corresponding to mild haemophilia. Regression analyses of the last year estimated rate of change in FVIII activity was -0.001 and -0.07 IU/dL/week for the 6 × 1013 and 4 × 1013 cohorts, respectively. Two participants (6 × 1013) resumed prophylaxis in year 7: one after a non-treatment-related G4 serious AE of spontaneous internal carotid artery bleed, and the other to manage bleeds and FVIII activity. CONCLUSIONS: The safety and efficacy of valoctocogene roxaparvovec remain generally consistent with previous reports, with good haemostatic control for most participants. Two participants returned to prophylaxis.

A Case of Cerebral Infarction due to Internal Carotid Artery Occlusion Accompanied by Persistent Primitive Trigeminal Artery.

An 85-year-old woman with a history of diabetes mellitus became aware of motor weakness of the left lower extremity. Magnetic resonance imaging showed multiple small cerebral infarctions in the right corona radiata. Angiography revealed persistent primitive trigeminal artery (PPTA) originating from the right internal carotid artery (ICA) and connecting to basilar artery, and the right ICA occluded distal to PPTA. Collateral blood circulation had developed, and sufficient collateral blood flow was expected. From these findings, the right ICA was considered to show stenosis due to atherosclerotic changes before occlusion. Conservative treatment was conducted with the transoral administration of rivaroxaban. It is important to correctly diagnose the anatomy and existence of an anomalous vessel in a stroke patient, not only when endovascular treatment is planned, but also for conservative medical treatment. Rapid and accurate radiological examinations facilitate safe and effective treatment.

Salvage endoscopic nasopharyngectomy in locally recurrent carcinoma adjacent to the internal carotid artery-A case report.

Key Clinical Message: In cases adjacent to critical structures, such as the internal carotid artery, surgeons should meticulously explore the feasibility of surgery before declaring the neoplasm unresectable. Abstract: Salvage treatment for locally recurrent carcinoma of the nasopharynx constitutes a unique challenge. Surgery remains the gold standard treatment modality. Endoscopic nasopharyngectomy is considered a safe and feasible procedure overcoming the morbidities of an open surgery. Tumor adjacency to the internal carotid artery (ICA) is not an absolute contradiction for the endoscopic approach. Even in cases adjacent to critical structures, surgeons should meticulously explore the feasibility of surgery before declaring the neoplasm unresectable. We present the case of a 56-year-old male with locally recurrent adenoid cystic carcinoma (AdCC) of the nasopharynx adjacent to the ICA treated with endoscopic nasopharyngectomy.

Natural History of Cavernous Carotid Artery Aneurysms: A Systematic Review and Meta-Analysis.

BACKGROUND: Cavernous carotid artery aneurysms (CCAAs) represent a common condition seen in clinical practice with significant practice variability. The aim of this systematic review and meta-analysis was to aggregate current evidence on the natural history of CCAAs. METHODS: MEDLINE/PubMed, EMBASE, and Cochrane Library were queried from inception until December 2023. The primary outcome of this study was CCAA-related mortality. The secondary outcomes of this study were aneurysm growth, intracranial ischemic and hemorrhagic events, improved non-cerebrovascular symptoms, and new or worsened non-cerebrovascular symptoms during follow-up. RESULTS: Ten studies met our inclusion criteria, involving 835 patients and 975 CCAAs. CCAA-related mortality had an incidence rate of 0.28 (95% confidence interval 0.12-0.64) per 100 person-years (PYs) of follow-up. The incidence rate of CCAA growth was 2.91 (1.05-8.07) per 100 PYs of follow-up. The incidence rate of CCAA-related intracranial ischemic events was 0.4 (0.16-1.01) per 100 PYs of follow-up. The incidence rate of CCAA-related intracranial hemorrhagic events was 0.54 (0.33-0.87) per 100 PYs of follow-up. The incidence rate of improved non-cerebrovascular symptoms was 2.51 (1.18-5.33) per 100 PYs of follow-up. The incidence rate of new or worsened non-cerebrovascular symptoms was 3.41 (2.03-5.73) per 100 PYs of follow-up. CONCLUSIONS: CCAAs are typically benign lesions with a low risk of rupture and life-threatening complications. CCAAs tend to follow an indolent course regarding non-cerebrovascular outcomes, and new or worsening symptoms are infrequent during the clinical course. However, spontaneous resolution of non-cerebrovascular symptoms and cranial nerve deficits at presentation is uncommon.

Treatment of an intracranial aneurysm in the setting of fenestration of cranial division of the internal carotid artery: Technical considerations and a literature review.

Although rare, cerebral arterial fenestration may present challenges in diagnosis and treatment. Here we present a case of a supraclinoid internal carotid artery (ICA) fenestration adjacent to an ICA aneurysm, successfully treated with balloon-assisted coil embolization. A female in her 50's presented with an acute subarachnoid hemorrhage from a ruptured left ICA-ophthalmic artery (OA) aneurysm. Digital subtraction angiography revealed a focal ICA fenestration distal to the posterior communicating artery (Pcom). The patient underwent successful coil embolization of the aneurysm using the balloon-assisted technique. No immediate hemorrhagic, thromboembolic, or neurological complications were observed. The patient was discharged in good condition after 2 weeks of hospitalization. A comprehensive literature review of 33 cases was subsequently performed to understand the characteristics of this condition. Cases involving the cranial division of the ICA forming the fenestration exhibited caliber differences significantly more frequently (p = 0.02). Embryological insights revealed distinctions between the cranial divisions of the ICA, influencing fenestration morphology and associated aneurysm formation. Endovascular treatment poses the risk of vascular injury, necessitating the identification of this variation and procedural planning.

Cavernous Sinus Syndrome in a Polio-Afflicted Patient With Multiple Aneurysms.

Cavernous sinus syndrome (CSS) is a complex, multifactorial condition that presents with a myriad of signs and symptoms including ptosis, double vision, and headache. We present the case of a 65-year-old woman with a chief concern of left-eye pain, including polio syndrome and hip replacement surgery. Unlike typical CSS cases often linked to tumors, this patient's condition involved a carotid-cavernous fistula (CCF), multiple internal carotid artery aneurysms, and a pericallosal aneurysm, without any associated tumor. She presented with severe left eye pain, ptosis, double vision, vomiting, headache, and other neurological symptoms since she woke up. Her treatment at a tertiary care center included diagnostic imaging, a cerebral angiogram, and embolization procedures, and she was discharged in stable condition. This case adds significant value to the medical literature by documenting the successful management of CSS with multiple aneurysms and a CCF, highlighting the importance of personalized treatment strategies and the effectiveness of modern embolization techniques in complex neurological conditions.

Successful endonasal transsphenoidal surgery to treat acute internal carotid artery occlusion caused by pituitary apoplexy: Usefulness of arterial spin labeling imaging for emergency decision.

Background: Pituitary apoplexy (PA) is a rare clinical condition presenting with acute headache, visual disturbance, and disorientation. PA can cause strokes due to acute internal cervical artery occlusion (ICO), which is an extremely rare condition. Arterial spin labeling (ASL) on magnetic resonance imaging (MRI) is a popular technique, which is a quantitative perfusion imaging useful for the diagnosis of ischemia. We report a treatment with acute pseudo-ICO in which ASL on MRI was useful for the decision of surgery timing. Case Description: A 50-year-old male presented with a sudden headache and nausea. MRI and magnetic resonance angiography revealed a large pituitary tumor and left ICO. However, the left middle cerebral and anterior cerebral arteries were depicted due to a cross-flow through the anterior communicating artery. ASL on MRI showed decreased perfusion of the left hemisphere, suggesting acute ICO. As he had no neurological deficit, we treated him conservatively, following the guidelines. Two days after admission, he presented with sensory aphasia and incomplete right paralysis. Emergency head computed tomography revealed a low-density area in his left temporal lobe. We decided on emergency tumor decompression surgery to prevent ischemic progression. We performed endonasal transsphenoidal surgery. Postoperative MRI showed recanalization of the left internal carotid artery (ICA). His incomplete right paralysis improved immediately after surgery but remains mild sensory aphasia. Conclusion: ICO-related PA is a very rare occasion but there are few similar reports. Some cases of successful ICO treatment due to PA have been reported, but the question of whether emergency or elective surgery is better remains unanswered. Our case may have been no neurological deficit if we had decided to have surgery on admission. Hypoperfusion of the ICA area due to PA may be an adaptation of emergency surgery. Perfusion images like ASL could be a useful technique to decide on surgery or conservative treatment.

Anatomical variations of the extracranial internal carotid artery: prevalence, risk factors, and imaging insights from CT-angiography.

PURPOSE: To determine the prevalence of different extracranial internal carotid artery (EICA) variations in CT angiography (CTA) of the neck and its predisposing factors. METHODS: In this retrospective study from 2021 to 2023 conducted in the radiology department of Shafa Hospital, Kerman, Iran, all patients who had undergone neck CTA were included. Expert radiologists blindly examined each CTA image for the following: EICA variations-coiling, kinking, straight morphology, and tortuosity-and the distance between the internal carotid artery and the apex of the epiglottis and the C2 lower margin. RESULTS: Of the 106 patients, the mean age was 55.9 ± 16.9 years. 64.2% were men, and 35.8% were women. Considering each patient's bilateral anatomy, the reported 70.28% (149/212) frequency of EICA variations of all arteries. Tortuosity, kinking, and coiling variation were found in 61.8%, 4.2%, and 4.2% of arteries, respectively. Also, 54.72%, 1.89%, and 0.94% of the participants had bilateral tortuosity, kinking, and coiling, respectively. There was a significant relationship between the prevalence of EICA variations and female sex, age, and hypertension. CONCLUSION: The frequency of EICA variations in arteries and patients was 70.28% and 73.58%, respectively. Tortuosity was the most common variation. Female sex, old age, and hypertension were significant risk factors for EICA variations.

A Rare and Dangerous Combination of COVID-19, Lemierre Syndrome, and Carotid Pseudoaneurysm: A Case Report.

Lemierre syndrome is a rare complication of oropharyngeal infection that causes septic thrombophlebitis in the internal jugular vein. Since the onset of the COVID-19 pandemic, this condition has been dangerously overlooked and poses an even greater threat when complicated by vascular pathologies. A case is presented where the patient required emergency endovascular exclusion of a right internal carotid artery pseudoaneurysm due to Lemierre syndrome. The treatment included stent graft placement and drainage of a neck abscess, along with appropriate antibiotic treatment during hospitalization. Recognizing this diagnosis requires a high index of suspicion, particularly during the COVID-19 pandemic. The complexity of the disease necessitates extensive multidisciplinary collaboration for effective treatment.

Effectiveness of S-Shaping of the Tip of the FUBUKI Guiding Catheter to Match the Shape of the Cervical Internal Carotid Artery in Anterior Circulation Cerebral Aneurysm Embolization.

Objective: During cerebral aneurysm embolization of the anterior circulation, the guiding catheter (GC) should be placed as distally as possible in the cervical internal carotid artery (ICA) to secure the maneuverability of the microcatheter and distal access catheter. However, if the shape of the tip of the GC does not appropriately match the course of the ICA, blood stasis might occur. We investigated whether shaping the tip of the GC into an S-shape would allow more stable catheterization to the distal ICA than the conventional GC with an angled tip. Methods: We included patients with cerebral aneurysms of the anterior circulation who were treated at our institution from April 2019 to April 2021. First, we evaluated the cervical ICA course in these patients through cerebral angiography and classified the courses into type S, type I, and type Z. Then, we focused on the most frequently encountered type-S cervical ICA to investigate the forging effect of the GC tip into an S-shape. We evaluated the lateral view of the carotid angiograms to examine whether the catheter tip reached the foramen magnum (FM) without interrupting ICA blood flow. The effects of age, sex, side, a history of hypertension and smoking, and an S-shape modification of the GC tip on the outcome of GC placement were analyzed. Results: A total of 67 patients were included in this study. The tip of the GC was placed at the FM in 27 cases. Among these factors, only the S-shape modification was significantly associated with whether the GC could be placed at the level of the FM (p <0.0001). Conclusion: By forging the tip of the GC into an S-shape, the GC can be safely advanced to the distal part of the cervical ICA, which may contribute to the improved maneuverability of microcatheters.

Cervical Spine Manipulation and Causation of Cervical Artery Dissection: A Review of 10 Case Reports.

Recent media coverage of high-profile cases of cervical artery dissection (CAD) has ignited the discussion about the role of cervical spine manipulation (CSM) in causing cervical artery dissection. However, research does not support a causal association between cervical spine manipulation and cervical artery dissection in a healthy cervical spine. The objective of this study was to review the 10 most recent case reports of cervical spine manipulation and cervical artery dissection for convincing evidence of the causation of cervical artery dissection by cervical spine manipulation. Nine of 10 case reports showed no convincing evidence of a causal relationship between cervical spine manipulation and cervical artery dissection. The 10th case report was exceptional as the CSM was contraindicated by pre-existing cervical spine pathology. We conclude that these 10 case reports provide no convincing evidence of the causation of cervical artery dissection by cervical spine manipulation in a healthy cervical spine. One case report demonstrated that cervical spine manipulation can cause cervical artery dissection when performed in the presence of pre-existing cervical spine pathology. Therefore, we conclude that practitioners should exclude cervical spine pathology before performing cervical spine manipulation.

The Relationship between the Permanent Trigeminal Artery and Cerebrovascular Disease: A Meta-Analysis.

Background: We aimed to analyze the variability of the permanent trigeminal artery (PTA) and its relationship with cerebrovascular disease. Methods: To analyze the variability of the PTA and its relationship with cerebrovascular disease by using the terms "primitive trigeminal artery", "persistent primitive trigeminal artery", " persistent trigeminal artery variant", "PPTA", "PTAV", "carotid- basilar anastomoses", "carotid-vertebrobasilar anastomoses", "persisting embryonic vessels" were used as keywords, and the English-language literature related to PTA and cerebrovascular diseases published in PubMed, EMBAS, and Web of Science databases from 2000 to 2022 were searched by using "subject terms + free words". A meta-analysis of the collected data was performed using stata14.0 statistical software to assess the relationship between the immortal trigeminal artery and cerebrovascular-related diseases. Results: A total of 1908 relevant articles were initially retrieved for this study. Ten papers were initially screened according to the inclusion and exclusion criteria, while the literature was then read one by one to exclude duplicates, reviews, case reports, and conference abstracts, and six papers were finally included for meta-analysis. The six papers included in this paper were all cross-sectional studies with 39,355 subjects, of which 206 subjects had PTA, with a variation rate of approximately 0.52%, including 77 males and 129 females, 117 left-sided variants and 87 right-sided variants. In contrast, of the 206 subjects with a PTA, 52 had cerebrovascular disease, with an overall prevalence of approximately 25.24%. Conclusion: The PTA could influence the development of cerebrovascular disease.

Ulceration location is associated with clinical course in carotid stenosis.

OBJECTIVE: Plaque ulceration in carotid artery stenosis is a risk factor for cerebral ischemic events; however, the characteristics that determine plaque vulnerability are not fully understood. We thus assessed the association between plaque ulceration sites and cerebrovascular ischemic attack. METHODS: We retrospectively collected the clinical data of 72 consecutive patients diagnosed with carotid artery stenosis with plaque ulcers. After excluding patients with pseudo-occlusion, a history of previous carotid endarterectomy or carotid artery stenting before the ulcer was first discovered, follow-up data of less than 1 month, or carotid endarterectomy or carotid artery stenting performed within 1 month after the ulcer was first discovered, 60 patients were ultimately included. Patients were divided into proximal and distal groups based on the ulcer location relative to the most stenotic point. The primary endpoints were ipsilateral cerebrovascular ischemic events ("ischemic events"), such as amaurosis fugax, transient ischemic attack, or ischemic stroke due to carotid artery stenosis with plaque ulceration. The association between ulcer location and ischemic events was also assessed. RESULTS: In the patients with plaque ulcer, more patients had proximal than distal plaque ulcers (39 vs 21; P = .028). The median follow-up duration was 3.8 years (interquartile range, 1.5-6.2 years). Nineteen patients (32%) experienced ischemic event. Ischemic events occurred more frequently in the distal than in the proximal group (18% vs 59%; P = .005). Kaplan-Meier curves demonstrated a significantly shorter event-free time in the distal group (log-rank P = .021). In univariate analysis, distal ulcer location was associated with ischemic events (odds ratio [OR], 2.94; 95% confidence interval [CI], 1.13-7.65; P = .03). Multivariate analysis using two different models also showed that distal ulcer location was independently associated with ischemic events (Model 1: OR, 3.85; 95% CI, 1.26-11.78; P = .03; Model 2: OR, 4.31; 95% CI, 1.49-12.49; P = .009). CONCLUSIONS: Patients with carotid artery stenosis and plaque ulcers located distal to the most stenotic point are more likely to experience cerebrovascular ischemic attacks. Therefore, carotid plaques with ulcers located distal to the most stenotic point may be a potential indication for surgical treatment.

An extremely rare complication during carotid artery stenting using a Wallstent: Migration of a stent holder of a Carotid Wallstent.

BACKGROUND: Carotid artery stenting (CAS) is an established treatment for internal carotid artery stenosis (ICS). The Carotid Wallstent is commonly used as a stent device in CAS procedures. Rare complications associated with the use of the Carotid Wallstent in CAS procedures include delayed shortening and incomplete stent expansion due to displacement of a marker ring. However, there have been no previous reports of a stent holder becoming unpredictably detached from a Carotid Wallstent during a procedure, requiring the additional deployment of another Carotid Wallstent. CASE PRESENTATION: The case of a 72-year-old man with progressive ICS is described. Since the ICS was resistant to medical treatment, CAS was planned to prevent ischemic events. During the procedure, a Mo.Ma Ultra and a carotid guardwire were used for proximal and distal protection, respectively. Following the introduction of those protective devices, a Carotid Wallstent was deployed at the stenotic lesion of the ICS. After the deployment of the Carotid Wallstent, a stent holder unpredictably detached was identified in the lumen of the Carotid Wallstent. Since it was impossible to mechanically retrieve the detached stent holder, another Carotid Wallstent was deployed to trap the substance. Postoperatively, the substance was identified as a stent holder of the first Carotid Wallstent. CONCLUSION: This is the first report of an extremely rare complication concerning a Carotid Wallstent in CAS.

The relationship between bone health and type of intracranial internal carotid calcifications in patients with ischemic stroke.

INTRODUCTION: Vascular calcifications, primarily in the aorta and its proximal branches, are commonly observed among subjects with impaired bone health. In this study, we sought to determine if a comparable association holds true for the calcifications in the intracranial internal carotid arteries (IICA), in general and also for particular calcification patterns. METHODS: A consecutive series of ischemic stroke patients were prospectively enrolled into the study, where computed tomography angiography source images were used to determine the presence and type of IICA calcifications, and dual-energy X-ray absorptiometry was used to determine the bone mineral density in the left femoral neck region. IICA calcifications were categorized as none, intimal, medial, and mixed types based on previously validated classification schemes. Their relationships with femoral bone T-scores were evaluated by bivariate and multivariate analyses. RESULTS: Femoral neck T-score was highest among patients without any vascular calcifications (n=65), when compared to the bone density measures among patients with any type of calcification (n=185) (p<0.001). After adjustment for age, gender, vascular risk factors, and serum biomarkers related to bone health, the T-score remained significantly associated only with the pattern of intimal calcification [OR 0.63 (0.42 - 0.95), p=0.028]. CONCLUSIONS: Our findings suggest that the intracranial vasculature, in particular the internal carotid arteries, is not immune to the interplay between suboptimal bone health and vascular calcifications. This association was most robust for an intimal type of IICA calcification pattern, while no such relationship could be demonstrated for other types of vascular calcifications.

[An anatomical road map of the parapharyngeal internal carotid artery in lateral and endoscopic ventral approaches].

Objective:To describe the road map of the lateral and endoscopic ventral approaches for the pharyngeal segment of the internal carotid artery, propose a sub-segmentation scheme, systematically and comprehensively understand its anatomical details and relationships with the surrounding structures. Methods:Five fresh cadaveric head specimens（10 sides in total） were dissected through lateral and endoscopic ventral approaches to evaluate the anatomical details of the parapharyngeal internal carotid artery and its relationship with the surrounding structures. Results:From the bifurcation of the common carotid artery to the vertical part of the internal carotid artery, alongside the direction of blood flow, the parapharyngeal internal carotid artery passes through four distinct anatomical tissues. Based on this, the parapharyngeal internal carotid artery can be divided into four sub-segments: nerve, muscle, fascia and osseous sub-segments. The boundaries and important adjacent structures of each segment are described in detail. Conclusion:The anatomical road map of the parapharyngeal internal carotid artery and the sub-segmentation scheme serving as a practical guide to navigate modular endoscopic skull base surgery of the parapharyngeal space while reduce the risk of internal carotid artery injury.

[Research progress in the anatomy and surgical approach of the parapharyngeal space].

The parapharyngeal space, a complex fascial compartment within the head and neck region, encompasses crucial anatomical structures including blood vessels and nerves. Tumors occurring within this space are rare, with the majority being benign in nature. Surgical intervention remains the primary treatment modality; however, managing parapharyngeal space tumors poses significant challenges due to their intricate anatomical configuration. Conventional open surgical approaches have been associated with significant tissue damage and a high prevalence of postoperative complications. Recently, advancements in anatomical studies and surgical techniques have led to significant progress in understanding parapharyngeal space anatomy and improving surgical management. This article aims to provide a comprehensive overview of these developments.