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Purpose: Understanding of rare or unknown anatomical variations of the vasculature of the neck is critical to reduce the risk of complications during surgeries and other invasive procedures in the neck and shoulder regions., Methods: Bilateral dissection of the neck and muscles of the back of an 87-year-old Caucasian male donor was performed to demonstrate the origin, course and termination of the arteries that arise in the neck., Results: Several anatomical variations were noted on the right side of the neck of the donor body - (i) only inferior thyroid and ascending cervical arteries originated from the thyrocervical trunk (TCT), from the first part of the subclavian artery (SA), whereas the transverse cervical (TCA) and suprascapular (SSA) arteries were entirely absent, (ii) Dorsal scapular artery (DSA) emerged normally from the third part of the SA. However, after supplying the rhomboids and levator scapulae muscles, DSA provided two additional branches to the trapezius muscle and a branch to the supraspinatus muscle. Interestingly, the branches to the trapezius muscle from the DSA were the only sources of blood supply to the muscle., Conclusion: We report a unique anatomical variation involving the absence of the TCA and SSA from the TCT. The unilateral absence of these major vessels and the branches of DSA supplying the trapezius and supraspinatus muscles have not been reported previously in the literature in a single case report. This case study may provide useful information for head and neck reconstruction and shoulder repair surgeries., (© 2024. The Author(s).)

Background: Arterial tortuosity syndrome is a rare Autosomal recessive disease that leads to a loss of function of the connective tissues of the body, this happens due to a mutation in the solute carrier family 2 member 10 (SLC2A10) gene. ATS is more likely to occur in Large and medium-sized arteries including the aorta and pulmonary arteries. This syndrome causes the arteries to be elongated and tortuous, This tortuosity disturbs the blood circulation resulting in stenosis and lack of blood flow to organs and this chronic turbulent flow increases the risk of aneurysm development, dissection and ischemic events., Case Presentation: A 2 years old Arabian female child was diagnosed with ATS affecting the pulmonary arteries as a newborn, underwent a pulmonary arterial surgical reconstruction at the age of 2 years old due to the development of pulmonary artery stenosis with left pulmonary artery having a peak gradient of 73 mmHg with a peak velocity of 4.3 m/s and the right pulmonary artery having a peak gradient of 46 mmHg with a peak velocity of 3.4 m/s causing right ventricular hypertension. After surgical repair the left pulmonary artery has a peak pressure gradient of 20 mmHg, with the right pulmonary artery having a peak pressure gradient of 20 mmHg., Conclusion: ATS is a rare genetic condition that affects the great arteries especially the pulmonary arteries causing stenotic and tortuous vessels that may be central branches or distal peripheral branches that leads to severe right ventricular dysfunction and hypertension. We believe that surgical treatment provides the optimum outcomes when compared to transcather approaches especially when the peripheral arteries are involved. Some challenges and hiccups might occur, especially lung reperfusion injury that needs to be diagnosed and treated accordingly., (© 2024. The Author(s).)

Cervicocerebral artery dissection stands out as a significant contributor to ischemic stroke in young adults. Several studies have shown that arterial tortuosity is associated with dissection. We searched Pubmed and Embase to identify studies on the association between arterial tortuosity and cervicocerebral artery dissection, and to perform a review on the epidemiology of cervicocerebral artery tortuosity and dissection, pathophysiology, measurement of vessels tortuosity, strength of association between tortuosity and dissection, clinical manifestation and management strategies. The prevalence of tortuosity in dissected cervical arteries was reported to be around 22%-65% while it is only around 8%-22% in non-dissected arteries. In tortuous cervical arteries elastin and tunica media degradation, increased wall stiffness, changes in hemodynamics as well as arterial wall inflammation might be associated with dissection. Arterial tortuosity index and vertebrobasilar artery deviation is used to measure the level of vessel tortuosity. Studies have shown an independent association between these two measurements and cervicocerebral artery dissection. Different anatomical variants of tortuosity such as loops, coils and kinks may have a different level of association with cervicocerebral artery dissection. Symptomatic patients with extracranial cervical artery dissection are often treated with anticoagulant or antiplatelet agents, while patients with intracranial arterial dissection were often treated with antiplatelets only due to concerns of developing subarachnoid hemorrhage. Patients with recurrent ischemia, compromised cerebral blood flow or contraindications for antithrombotic agents are usually treated with open surgery or endovascular technique. Those with subarachnoid hemorrhage and intracranial artery dissection are often managed with surgical intervention due to high risk of re-hemorrhage., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

The effect of arterial tortuosity on intracranial atherosclerosis (ICAS) is not well understood. This study aimed to evaluate the effect of global intracranial arterial tortuosity on intracranial atherosclerotic burden in patients with ischemic stroke. We included patients with acute ischemic stroke who underwent magnetic resonance angiography (MRA) and classified them into three groups according to the ICAS burden. Global tortuosity index (GTI) was defined as the standardized mean curvature of the entire intracranial arteries, measured by in-house vessel analysis software. Of the 516 patients included, 274 patients had no ICAS, 140 patients had a low ICAS burden, and 102 patients had a high ICAS burden. GTI increased with higher ICAS burden. After adjustment for age, sex, vascular risk factors, and standardized mean arterial area, GTI was independently associated with ICAS burden (adjusted odds ratio [adjusted OR] 1.33; 95% confidence interval [CI] 1.09-1.62). The degree of association increased when the arterial tortuosity was analyzed limited to the basal arteries (adjusted OR 1.48; 95% CI 1.22-1.81). We demonstrated that GTI is associated with ICAS burden in patients with ischemic stroke, suggesting a role for global arterial tortuosity in ICAS., (© 2024. The Author(s).)

Arterial tortuosity syndrome is an extremely rare hereditary connective tissue disorder. We present a case of an incidentally diagnosed aneurysm of the aortic root and the ascending aorta caused by arterial tortuosity syndrome, which was confirmed genetically. The aneurysm was repaired surgically. One year after the procedure, there was no further dilation of the aorta or formation of new aneurysms.

Introduction: The accessory appendicular artery (AAA) is an accessory source of blood supply to the appendix. Its existence and potential point of origin are seldom addressed in the literature., Methods: To fill this knowledge gap, we performed a systematic review of all available studies involving both cadaveric and surgical specimens and documented the presence and the origin of the AAA, following the guidelines outlined in the preferred reporting items for systematic reviews and meta-analysis (PRISMA) statement., Results: Eleven studies, with an overall of 604 specimens were included. Our research revealed that the AAA is notably absent in most cases (83.6%). When present, it most commonly originates from the posterior cecal artery (12.4%), followed by the descending branch of the ileocolic artery (2%), and the ileal branch of the ileocolic artery (0.7%). Instances of origin from the anterior cecal, common cecal, or the ileocolic trunk were even more infrequent. Based on our observations, we introduced a new simplified classification system., Discussion: The effect of an accessory artery on the process of appendicitis remains to be clarified. We firmly recommend that surgeons should consider the possible presence and various origins of the AAA during appendectomy procedures to avoid serious complications., (© 2024 Royal Australasian College of Surgeons.)

In most cases, the superior laryngeal artery (SLA) branches from the superior thyroid artery, which, in turn, leaves the external carotid artery. Few dissection studies found previously that the SLA could originate from the lingual artery. We report here probably the first evidence of such a rare anatomical variation found unilaterally in a retrospectively evaluated by computed tomography angiography adult male case. The left SLA left a suprahyoid coil of the lingual artery and continued over the greater hyoid horn to enter the larynx through the thyrohyoid membrane. On both sides, thyroid foramina were found, but only the right one used for the entry of the right SLA. Therefore, the rare SLA origin from the lingual artery can be documented on computed tomography angiograms, which could help during preoperative evaluations and prevent unwanted surgical complications., (© 2024. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

Loeys-Dietz syndrome (LDS) is an autosomal connective tissue disorder commonly presenting with hypertelorism, bifid uvula, aortic aneurysms, and arterial tortuosity. The aim of the present study was to investigate differences in tortuosity index (TI) between genotypes of LDS, possible progression over time and its use as an adjunctive prognostic tool alongside aortic dimensions to aid timely surgical planning in pediatric patients. A retrospective observational study of pediatric LDS patients referred to our center (November 2012-February 2021) was conducted. Using magnetic resonance angiography (MRA) with 3D maximum intensity projection volume-rendered angiogram, arterial TI was measured. Twenty three patients had genetically confirmed LDS with at least one head and neck MRA and 19 had no less than one follow-up MRA available. All patients presented arterial tortuosity. Patients with TGFBR2 variants had greater values of TI compared to patients with TGFB2 variants (p = 0.041). For patients who did not undergo surgery (n = 18), z-scores at the level of the sinus of Valsalva showed a significant correlation with vertebral TI (r s  = 0.547). There was one death during follow-up. This study demonstrates that patients with LDS and TGFBR2 variants have greater values of TI than patients with TGFB2 variants and that greatest values of TI are associated with increased aortic root z-scores. Furthermore, as TI decreases over time, less frequent neuroimaging follow-up can be considered. Nevertheless, additional studies are needed to better define more accurate risk stratification and long-term surveillance in these patients., (© 2023 Wiley Periodicals LLC.)

Systemic arterial blood supply to a normal lung is a rare anatomical abnormality. Surgery is usually indicated because this abnormality leads to pulmonary hypertension. Herein, we report our experience and ideas for safe vessel dissection. Case 1 was a woman in her 50s. We performed a left lower lobectomy following percutaneous coil embolization. The aberrant artery with emboli was confirmed intraoperatively by cone-beam computed tomography (CBCT) to safely dissect under thoracoscopic surgery (TS). Case 2 was a man in his 40s. Following percutaneous endovascular plug occlusion, we performed a left partial resection using indocyanine green fluorescence navigation. Intraoperatively, CBCT imaging demonstrated the aberrant artery and exact position of the emboli. This combination technique of interventional radiology and TS with CBCT imaging was considered safe and more secure for the treatment of anomalous systemic arterial blood supply to a normal lung.

A personalized approach with attention to anamnesis and specific symptoms is necessary in patients with internal carotid artery tortuosity. Neuroimaging (especially before elective surgery) or functional stress tests following ultrasound of supra-aortic vessels may be necessary depending on medical history and complaints. In addition to standard Doppler ultrasound, these patients should undergo rotational and orthostatic transformation tests. We analyze changes in shape and hemodynamic parameters within the tortuosity area in various body positions. This is especially valuable for patients with concomitant carotid artery stenosis. The article presents a clinical case illustrating the importance of such approach.

The review is devoted to diagnosis and treatment of internal carotid artery tortuosity. The authors consider modern classification, epidemiology and diagnostic options using neuroimaging or ultrasound-assisted functional stress tests depending on medical history and complaints. In addition to standard Doppler ultrasound, rotational and orthostatic tests are advisable due to possible changes of local shape and hemodynamic parameters following body position changes, especially in patients with concomitant atherosclerotic stenosis. Thus, a personalized approach is especially important for treatment and diagnostics of internal carotid artery tortuosity.

A persistent stapedial artery is a congenital vascular malformation that can cause tinnitus and/or conductive hearing loss. Although rare, this case highlights the importance of recognizing aberrant anatomy as a potential cause of patients' symptoms. It also demonstrates how to recognize and treat patients with a symptomatic persistent stapedial artery., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Many researchers have found a correlation between tortuous arteries and development of aneurysms in cerebral arteries. We decided to determine whether tortuosity of the internal carotid artery can be related to its aneurysm rupture., Methods: We retrospectively analyzed the internal carotid artery anatomy of 149 patients with internal carotid artery aneurysms. For each patient, we calculated relative length (RL), sum of angle metrics (SOAM), triangular index (TI), product of angle distance (PAD), and inflection count metrics (ICM)., Results: A total of 33 patients (22.15%) had subarachnoid hemorrhage. These patients had significantly lower SOAM (0.31 ± 0.17 vs. 0.42 ± 0.21; P < 0.01), TI (0.27 ± 0.09 vs. 0.31 ± 0.11; P = 0.03) and ICM (0.25 ± 0.11 vs. 0.31 ± 0.17; P = 0.04). In multivariate logistic regression analysis, higher SOAM (odds ratio, 0.780; 95% confidence interval, 0.619-0.961; P = 0.025) remained independently associated with lower risk of internal carotid artery aneurysm rupture. In addition, we found significant positive correlation of aneurysm dome size with SOAM (R = 0.224; P = 0.013) and PAD (0.269; P < 0.01). Our study also showed that age (R = 0.252; P = 0.036), Glasgow Coma Scale score (R = -0.706; P < 0.01), and TI (R = -0.249; P = 0.042) were independently correlated with modified Rankin Scale score on discharge., Conclusions: Lower tortuosity might be a protective factor against internal carotid artery aneurysm rupture and poor outcome after subarachnoid hemorrhage. Higher tortuosity is correlated with internal carotid artery aneurysm growth., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: To investigate the association of middle cerebral artery (MCA) bifurcation aneurysms with bifurcation morphology., Materials and Methods: 205 patients were enrolled, including 61 patients with MCA bifurcation aneurysms and 144 non-aneurysmal subjects. Aneurysmal cases were divided into types C (aneurysm neck on extension of the parent artery centerline) and D (deviating neck). The radius of the parent artery M1 (R P ) and bilateral branches (R S and R L , respectively), smaller (φ S ) and larger (φ L ) lateral angles, bifurcation angle, and arterial tortuosity from parent vessel to bilateral branches (T S and T L , respectively) were analyzed. Logistic regression and receiver operator characteristic (ROC) curve analysis were performed to identify risk factors and predictive values for MCA aneurysm presence and types., Results: In aneurysmal MCA bifurcations, bifurcating angle, T S , T L and R L were significantly larger (P<0.01), while φ S was significantly smaller (P<0.001) than those in controls. The bifurcation angle, T S and LogitP were better morphological parameters for predicting MCA aneurysm presence with the AUC of 0.795, 0.932 and 0.951, respectively. Significant (P<0.05) differences were observed in the bifurcation angle, φ L , R P , R L and T L between types C and D aneurysmal bifurcations. T L was an independent factor in discriminating types C from D aneurysms with an AUC of 0.802., Conclusions: Bifurcation angle and arterial tortuosity from the parent artery to the branch forming a smaller angle with the parent artery have a higher value in distinguishing MCA aneurysmal from non-aneurysmal ones, and the tortuosity from the parent artery to the contralateral branch is the best indicator for distinguishing types C from D aneurysmal bifurcations., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Arterial tortuosity syndrome (ATS) is a rare autosomal recessive disease characterized by elongation and tortuosity of the large- and medium-sized arteries. ATS patients display features that are also found in Ehlers-Danlos syndrome (EDS) patients. ATS is caused by pathogenic mutations in the SLC2A10 gene, which encodes for the glucose transporter, GLUT10. This study aimed at examining the ultrastructure of skin for abnormalities that can explain the loose skin and arterial phenotypes of Arab patients with the p.S81R mutation in SLC2A10. Forty-eight patients with SLC2A10 mutation were recruited for this study. Skin biopsy specimens from three children with ATS and a healthy child were examined by electron microscopy to determine the ultrastructure of collagen and elastin. Histopathologic staining of sections from tissue biopsy specimens was also performed. Large spaces were observed among the collagen fibrils in the skin biopsy specimens obtained from ATS patients, suggesting disorganization of the collagen structures. Furthermore, elastin fiber contents and their thickness are reduced in the skin. In small muscular arteries in the skin from ATS patients, discontinuous internal elastic lamina, lack of myofilaments, and disorganized medial smooth muscle cells with vacuolated cytoplasm are present. The disorganization of collagen fibrils and reduced elastin contents in the skin may explain the loose skin phenotype of ATS patients similar to the EDS patients. The lack of elastin in small muscular arteries may have contributed to the development of arterial tortuosity in these patients., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Arterial tortuosity syndrome is a rare hereditary autosomal recessive connective tissue disorder characterized by elongation and tortuosity of the large- and medium-sized arteries. We present the case of a 13-year old child with arterial tortuosity syndrome who underwent occipital encephaloduroarteriomyosynangiosis for posterior circulation insufficiency. The constellation of clinical features in our patient portended significant anesthetic concerns, including difficult airway due to craniofacial abnormalities, risk of stroke, and myocardial infarction due to intracerebral and coronary arterial tortuosity and stenosis. The pertinent anesthetic implications are summarized, and we describe the anesthetic technique and use of multimodal neuromonitoring relevant for the case., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 International Anesthesia Research Society.)

Background: Surgical pulmonary artery reconstruction in patients with arterial tortuosity syndrome has excellent outcomes. In this study, we report our late outcomes after more than a decade of experience with such complex interventions., Methods: We conducted a retrospective review of 33 arterial tortuosity syndrome patients who underwent pulmonary artery reconstruction. The mean preoperative right ventricular to left ventricular pressure ratio was 1.19 ± 0.2. Our surgical approach included either a single-stage complete repair through a median sternotomy (17 patients) or a two-stage repair through sternotomy/left thoracotomy (16 patients), depending on the degree of distal involvement in the left pulmonary artery., Results: Median age was 36 months. All patients had distal segmental peripheral pulmonary artery stenosis. Thirty patients (90.1%) were symptomatic before surgery. There was one hospital death due to viral pneumonia 78 days after the surgery (in-hospital mortality 3%). The mean right ventricular to left ventricular pressure ratio decreased to 0.31 ± 0.07 early postoperatively (P < 0.001), representing a 74% reduction compared with preoperative values. Follow-up was 100% complete for all hospital survivors (32 of 33) with a mean follow-up of 70.42 ± 43.32 months (range, 2 to 143). There was no late mortality or need for reintervention (surgical or catheter based) after hospital discharge. In late postoperative catheterization, the mean right ventricular to left ventricular pressure ratio was 0.27 ± 0.05 (P = .003 compared with early postoperative value). All patients were asymptomatic on their most recent follow-up., Conclusions: A strategy of complete surgical reconstruction of all stenotic pulmonary artery segments in patients with arterial tortuosity syndrome is recommended for sustainable successful outcomes more than a decade later., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Introduction: The association between arterial tortuosity and acute ischemic stroke (AIS) has been reported, but showing inconsistent results. We hypothesized that tortuosity of extra- and intracranial large arteries might be higher in AIS patients. Furthermore, we explored the correlation between artery tortuosity and white matter hyperintensity (WMH) severity in AIS patients., Methods: 166 AIS patients identified as large artery atherosclerosis, and 83 control subjects were enrolled. All subjects received three-dimensional computed tomography angiography (CTA). Arterial tortuosity was evaluated using the tortuosity index. WMHs were evaluated using magnetic resonance imaging in all AIS patients., Results: AIS patients showed significantly increased arterial tortuosity index relative to controls, including left carotid artery (CA) ( p = 0.001), right CA ( p < 0.001), left common carotid artery (CCA) ( p < 0.001), right CCA ( p < 0.001), left internal carotid artery ( p = 0.001), right internal carotid artery ( p = 0.01), left extracranial internal carotid artery (EICA) ( p < 0.001), right EICA ( p = 0.01), and vertebral artery dominance (VAD) ( p = 0.001). The tortuosity of all above arteries was associated with the presence of AIS. AIS patients with moderate or severe WMHs had a higher tortuosity index in left CA ( p = 0.005), left CCA ( p = 0.003), left EICA ( p = 0.07), and VAD ( p = 0.001). In addition, the tortuosity of left EICA and VAD was associated with WMH severity in AIS patients., Conclusions: Increased extra- and intracranial large arteries tortuosity is associated with AIS. The tortuosity of left carotid artery system and vertebral artery may be the independent risk factors for WMH severity in AIS patients. Clinical Trial Registration . This trial is registered with NCT03122002 (http://www.clinicaltrials.gov)., Competing Interests: The authors declare that there is no conflict of interest., (Copyright © 2022 Ke Shang et al.)

Background: Anomalous systemic arterial supply to normal basal lung segments is a rare congenital malformation, in which aberrant arteries arising from the systemic circulation flow into the basal segment of the lung and return to normal pulmonary veins without abnormal bronchial branching. It presents a left-to-right shunt, resulting in volume overload of the pulmonary circulation, and consequently, pulmonary hypertension. Therefore, nearly all cases require surgery. Herein, we present a case, in which indocyanine green was used to demarcate the lung segment perfused by an anomalous systemic artery., Case Presentation: A 15-year-old boy was diagnosed with an anomalous artery originating from the celiac artery and supplying the right dorsobasal lung segment (S 10 ). Via three-port video-assisted thoracoscopic surgery, the anomalous artery was ligated and processed with an auto-stapler. Indocyanine green was injected intravenously to identify the tissue perfused by the anomalous artery, and the lung was resected., Conclusions: With anomalous systemic arterial supply to normal basal lung segments, indocyanine green can be particularly helpful in identifying the boundaries of the perfused area. Then, the affected tissue can be resected by thoracoscopic surgery., (© 2022. The Author(s).)

The aim of this article is to describe neurovascular findings in patients with Loeys Dietz syndrome type III and their possible clinical impact. Loeys Dietz syndrome type III, caused by pathogenic SMAD3 variants, is an autosomal dominant syndrome characterized by aneurysms and arterial tortuosity in combination with osteoarthritis. Neurovascular abnormalities have been described in other heritable aortic syndromes, however, reliable data in Loeys Dietz syndrome type III is missing. In our tertiary center, all adult patients with confirmed Loeys Dietz syndrome type III are followed in a standardized aorta outpatient clinic including Computed Tomography Angiography (CTA) of the head and neck region at baseline and (tri) yearly during follow-up. We performed an analysis of the neurovascular imaging findings and clinical follow-up. The primary outcome was a combined endpoint of mortality, dissection, cerebral vascular event and intervention. In addition, tortuosity and vascular growth were assessed. In total 26 patients (mean age 38.4 years, 38.5% males) underwent 102 (mean 3.9 (1-8) per patient) neurovascular Computed Tomography Angiography scans between 2010 and 2021. In 84.6% some form of neurovascular abnormality was found. The abnormalities at baseline were aneurysm (26.9%) dissection flap (7.7%), arterial tortuosity (61.5%), arterial coiling (23.1%) and arterial kinking (3.8%). During follow up (mean 8.85 (1-11) years) one patient suffered from sudden death and one patient needed a neuro-radiological intervention. No cerebral bleeding or stroke occurred. In conclusion, neurovascular imaging in Loeys Dietz syndrome type III patients revealed abnormalities such as aneurysm, tortuosity, coiling and kinking in the vast majority of patients, but clinical events were rare. Neurovascular screening and follow up is advised in all Loeys Dietz syndrome type III patients., (Copyright © 2022 The Authors. Published by Elsevier Masson SAS.. All rights reserved.)

Objectives: The purpose of this article is to review the embryology of the lower limb arterial anatomy along with common variants and their clinical relevance., Design: Embryologic variations of the lower limb arterial system may be explained by i.) persistence of primordial arterial segments, ii.) abnormal fusion, iii.) segmental hypoplasia/absence, or a combination of both. Persistent sciatic artery, corona mortis, and popliteal entrapment syndrome will also be discussed with associated symptoms, and potential complications., Conclusion: Knowledge of these variations is essential for surgical and endovascular management as failure to recognize them can result in complications.

The aim of our study was to evaluate the association between intracranial arterial tortuosity and cardiovascular outcome in patients with Loeys-Dietz syndrome (LDS). We performed a retrospective analysis of all patients with genetically confirmed LDS who underwent at least one brain MRA at our institution (n = 32); demographic and clinical features were evaluated in relation to the tortuosity of intracranial arteries as measured by tortuosity index (TI), which was calculated using the formula: [(centerline length) / (straight-line length)-1] × 100. Receiver operating characteristic curve analysis for intracranial TI and the binary end point of aortic surgery showed vertebrobasilar TI (VBTI) to be the best classifier among the examined arterial segments (AUC = 0.822). Patients with higher VBTI showed a greater incidence of aortic surgery (p < 0.001) and underwent more surgical and endovascular procedures (p = 0.006), with a higher rate of operations (p = 0.002). Kaplan-Meier analysis showed a significantly longer surgery-free survival in patients with lower arterial tortuosity (p < 0.001). At multivariate analysis, higher VBTI was associated with an increased risk of surgery (p < 0.001), which was independent of gene mutation and patient age. Increased VBTI is a marker of adverse cardiovascular outcome in patients with LDS, which can be easily measured on brain MRA, and may be useful in the management of this heterogeneous patient population., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Arterial tortuosity describes variation via bending of the arterial wall and has been noted in several arteries throughout the body. Tortuous blood vessels can cause nerve compression, as well as present difficulties to surgeons and radiologists. Here we present an unusual case of multi-vessel arterial tortuosity discovered in 78-year-old Hispanic male cadaver, independent of systemic pathology. The left ulnar and right tibial arteries were dissected, and using calibrated digital callipers, their external and internal diameters were measured both at the origin site and at the site of greatest dilation. Both wall thickness and the number of inflection points were also measured. Six bends were noticed in the ulnar artery and its diameter measured 8.11 mm at its widest, with a wall thickness of 0.88 mm. On the lower extremity, the right tibial artery had three bends and its diameter measured 4.86 mm at its widest, with a wall thickness of 1.32 mm. This uncommon tortuosity is not only more prone to laceration during surgery, but the bending and thickening can be mistaken for tumours. Finally, fluid dynamics can be altered, resulting in an impact on blood pressure in the extremities. Thus, raising awareness is crucial to prevent both symptoms and iatrogenic complications.

Objective: Pulsatile tinnitus (PT) caused by an aberrant artery is rare. We report an unprecedented cause of PT resulting from an aberrant artery coursing the mastoid surface, and qualitatively discuss the pathophysiology of PT., Methods: This case study reports a 41-year-old woman who presented with persistent PT at her right retromastoid region. Contrast-enhanced computed tomography revealed an aberrant branch of the artery that coursed over the cortex of the mastoid bone. Surgical ligation of this aberrant artery was performed under local anesthesia., Results: Intraoperative findings suggested that PT transmitted via bone-conduction route due to the direct contact of the vascular and mastoid surface. PT was completely resolved upon surgical removal of this causative segment. Ultrasonographic and hemodynamic analysis showed that the turbulent kinetic energy and high regional wall pressure were the major contributory factors causing PT. Spectro-temporal analysis showed that PT fluctuates at frequency 500~2000 Hz, which differs from those of venous PT., Conclusion: Judicious preoperative and intraoperative assessments of PT ensure the surgical efficacy of PT. Objective ultrasonographic and computational studies can provide detailed hydroacoustic characteristics of PT., Competing Interests: Declaration of Competing Interest There are no financial disclosures or conflicts of interests for any of the above named authors., (Copyright © 2020. Published by Elsevier B.V.)

Background: Fibular flaps have been widely used for mandibular and maxillary reconstructions. On occasion, anatomical variants of fibular arteries (FA) will be encountered., Purpose: Although anatomical variants of FA during fibular harvest have been reported, controversy exists regarding whether simple color Doppler ultrasonography (CDU) and physical examinations (PE) are sufficient for early preoperative detection., Material and Methods: A 10-year retrospective analysis in our department was performed to find the patients with various FA anomalies confirmed by computed tomography angiography (CTA) or intraoperative findings., Results: A total number of 19 FA anomalies were found either pre- or intraoperatively in 16 patients, in whom three cases were with bilateral FA variants. Type IIIC variants, also called arteria peronea magna (great peroneal artery), were confirmed in two legs, while the majority (13 legs) had type IIIA hypoplastic/aplastic posterior tibialis arteries (PTA). Four legs had new type IIID (low FA and PTA bifurcations). Preoperative CDU and PE only suspected anomalies in two legs. Six cases proceeded with using the affected fibulas, within whom vascular grafts were used in half of them for lengthening the FA pedicle. Local ischemia, partial soleus muscle necrosis, and claudication were reported in one., Conclusions: Routine CTA before every fibular harvest, rather than simple PE and CDU, should be added for screening contraindications and ensuring safety for fibular flap harvest.

Background: Arterial Tortuosity Syndrome (ATS) is a rare autosomal recessive disorder characterized by elongated and tortuous arteries. Although ATS showed a significant clinical and pathophysiological overlap with other syndromes involving connective tissues, only few cases of cerebrovascular events related to this syndrome have been described so far., Case Presentation: We report the case of a 33-years-old male diagnosed with ATS since childhood, that experienced three sudden episodes of expressive aphasia and right hemiparesis with spontaneous resolution. He was treated with recombinant tissue plasminogen activator (r-TPA) at a dosage of 0.9 mg/kg with a complete recovery. Brain Magnetic Resonance Imaging (MRI) showed the absence of acute ischemic lesions and the patient was diagnosed with recurrent transient ischemic attacks (TIA). Intracranial and supra-aortic trunks Magnetic Resonance Angiography (MRA) and Angio-CT scan of the thoracic and abdominal aorta showed marked vessel tortuosity without stenosis. To our knowledge, this is the first reported case of an ATS patient with TIA in young age that was treated with intravenous thrombolysis with recombinant plasminogen activator., Conclusion: Our report strengthens the relationship between ATS and juvenile cerebrovascular events, suggesting that an extensive study of body vessels in order to detect potential stenoses or occlusions in these cases is needed. The greater predisposition to cerebrovascular events in ATS could benefit from a more aggressive primary and secondary prevention therapy., (© 2021. The Author(s).)

Purpose: To explore the association between carotid artery length and tortuosity, and the occurrence of stroke., Material and Methods: In this retrospective study, IRB approved, 411 consecutive patients (males: 245; median age: 56 ± 12 years, age range: 21-93 years) with anterior circulation ischemic stroke were included. Only patients that underwent CTA within 7 days were considered and stroke caused by cardiac embolism and thoracic aorta embolism were excluded. For each patient, both carotid arteries were considered, and the ICA, CCA-ICA length and tortuosity were calculated. Inter-observer analysis was quantified with the Bland-Altman test. Mann-Whitney test and logistic regression analysis were also calculated to test the association between length and tortuosity with the occurrence of stroke., Results: In the final analysis, 166 patients (males: 72; median age: 54 ± 12 years, age range: 24-89 years) with anterior circulation ischemic stroke that were admitted to our hospital between February 2008 and December 2013 were included. The results showed a good concordance for the length of the vessels with a mean variation of 0.7% and 0.5% for CCA-ICA and ICA length respectively an for the tortuosity with a mean variation of 0.2% and -0.4% for CCA-ICA and ICA respectively. The analysis shows a statistically significant association between the tortuosity index of the ICA and CCA-ICA sides with stroke ( P value = 0.0001 in both cases) and these findings were confirmed also with the logistic regression analysis., Conclusion: Results of this study suggest that tortuosity index is associated with the presence of stroke whereas the length of the carotid arteries does not play a significant role.

Background: Dieulafoy's lesion (DL) is a rare but increasingly recognized cause of severe upper GI hemorrhage (SUGIH). There is little consensus regarding the endoscopic approach to management of bleeding from DL., Aims: Our purposes were to compare 30-day outcomes of patients with SUGIH from DL with Doppler endoscopic probe (DEP) monitoring of blood flow and guided treatment versus standard visually guided hemostasis (VG)., Methods: Eighty-two consecutive DL patients with SUGIH were identified in a large CURE Hemostasis database from previous prospective cohort studies and two recent RCTs at two university-based medical centers. 30-day outcomes including rebleeding, surgery, angiography, death, and severe medical complications were compared between the two treatment groups., Results: 40.2% of DL bleeds occurred in inpatients. 43.9% of patients had cardiovascular disease, and 48.7% were taking medications associated with bleeding. For the entire cohort, 41.3% (26/63) of patients treated with VG had a composite 30-day outcome as compared to 10.5% (2/19) of patients treated with DEP (p = 0.017). Rebleeding occurred within 30 days in 33.3% and 10.5% of those treated with VG and DEP, respectively (p = 0.051). After propensity score matching, the adjusted 30-day composite outcome occurred in 39.0% in the VG group compared to 2.6% in the DEP group (p < 0.001). Adjusted 30-day rebleeding occurred in 25.3% in the VG group versus 2.6% in the DEP group (p < 0.001)., Discussion: DL patients with SUGIH were frequently inpatients and had severe cardiovascular comorbidities and recurrent bleeding. Lesion arterial blood flow monitoring and obliteration are an effective way to treat bleeding from DL which reduces negative 30-day clinical outcomes., (© 2020. Springer Science+Business Media, LLC, part of Springer Nature.)

Purpose: This study aims to provide data, with the use of computed tomography angiography, regarding the level of bifurcation of the peroneal artery to the anterior perforating branch and the lateral calcaneal branch, in relation to the osseous anatomic structures of the tibial plafond, the medial malleolus and the lateral malleolus., Methods: The study included patients who underwent diagnostic computed tomography angiography of the lower extremities. Measurements were performed in two-dimensional reconstructions and included the perpendicular distance from peroneal artery bifurcation into anterior perforating branch and lateral calcaneal branch to the lowest level of tibial plafond (D1), medial malleolus (D2) and lateral malleolus (D3). The distances were also normalized to the length of the tibia., Results: Sixty patients and a total of 115 limbs were enrolled in this study. The mean distance ± standard deviation from peroneal artery bifurcation to tibial plafond (D1) was 4.33 ± 1.12 cm (normalized 0.12 ± 0.03) (range 2.54-8.26 cm), to medial malleolus (D2) was 5.53 ± 1.18 cm (normalized 0.16 ± 0.03) (range 3.27-9.5 cm) and to lateral malleolus (D3) was 6.53 ± 1.17 cm (normalized 0.18 ± 0.03) (range 4.71-10.2 cm), respectively. There was no significant difference between right and left limb measurements (p > 0.05). Females presented lower, but not statistically significant (p > 0.05), D1, D2 and D3 measurements compared to males., Conclusion: The bifurcation of the peroneal artery takes place at lower level compared to previously published studies and consequently extreme caution should be exercised when performing the posterolateral approach to the ankle. This study adds to the understanding of the relevant vascular anatomy of the region and assists in performing the posterolateral approach to the ankle with safety., (© 2021. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

Objective: Retrotransverse foramen (RTF) and retrotransverse groove (RTG) are anatomic variations of the atlas (C1) vertebrae. RTF contains an anastomotic vein connecting atlanto-occipital and atlanto-axodian venous sinuses. The purpose of this study was to analyze the arterial vascular structures running though the RTF and RTG., Methods: Three-dimensional volume rendered computed tomography angiography (3D VR CTA) images of 427 patients (264 men, 163 women; age 17-87 years) were reviewed and evaluated using the RadiAnt DICOM Viewer (version 5.0.2; Medixant, Poznan, Poland). The incidence of RTF or RTG, the incidence of the V3 segment of vertebral artery variants, and the artery vascular structures inside the RTF and RTG anatomic variation of C1 were analyzed., Results: Fifty (11.7%) atlases presented RTF anatomical variant; 113 (26.5%) atlases presented RTG anatomical variants. The incidence of the V3 segment of vertebral artery variants was 0.94% (4 of 427). Three (0.7%) were persistent first intersegmental artery and 1 (0.2%) was the fenestration of the vertebral artery on left side. In 4 cases of C1 vertebral artery V3 segmental variants, there were no RTF and RTG. No artery vascular structure was found in RTF or RTG., Conclusions: The RTF or RTG of C1 was a common anatomical variant. No arterial vascular structure runs though the RTF or RTG. The presence of C1 RTF and RTG variants had no effect on the V3 segmental course of the vertebral artery. Preoperative understanding of these variations using 3D CTA are helpful for the safe execution of the upper cervical posterior approach surgeries., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Longer intervals to reperfusion in patients treated with mechanical thrombectomy (MT) for emergent large vessel occlusion (ELVO) stroke are associated with worse outcomes and influenced by the operator's ability to navigate individual anatomy. Our aims were to assess the impact of time from puncture to first deployment of the MT device (DT) on technical and clinical outcomes, develop an A natomical A s sessment for M echanical T hrombectomy S core (ASMETS) that could predict DT and assess how different methods of intracranial access (coaxial-direct or exchange) influence this., Methods: Retrospective review of a prospective database of patients treated with MT for ELVO between November 2015 and August 2018. CTAs were assessed for ASMETS. Intracranial access technique was at the discretion of the operator. Technical and clinical outcomes and complications were recorded. Linear and logistic regression analysis was performed., Results: 92 patients were included. The impact of DT on clinical outcomes was significant. An unfavourable ASMET score is significantly associated with longer DT (p = 0.002) and linear regression showed DT time can be predicted by ASMETS - F(1,90) = 6.182, p = 0.015. No difference was demonstrated between different access techniques., Conclusion: CTA-based ASMETS can predict time between arterial puncture and deployment of the mechanical thrombectomy device in stroke patients, irrespective of the technique used to catheterise the target ICA. This could inform the operator in preparing appropriate strategies to overcome challenging vascular anatomy in patients undergoing MT.

Persistent median artery (PMA) has been recognized as a major arterial variant of the forearm in about 30% adults and it also shows a continuously increasing trend in the last two hundred years. This may explain well the mystery why a "new meridian" was added to the older 11-meridian system over two thousand years ago in Chinese medicine. The 12th meridian, Hand Jue Yin Meridian, happens to run along the same pathway as PMA., (© 2021 Anatomical Society.)

Significance: Cardiovascular disorders are the most important cause of morbidity and mortality in the Western world. Monogenic developmental disorders of the heart and vessels are highly valuable to study the physiological and pathological processes in cardiovascular system homeostasis. The arterial tortuosity syndrome (ATS) is a rare, autosomal recessive connective tissue disorder showing lengthening, tortuosity, and stenosis of the large arteries, with a propensity for aneurysm formation. In histopathology, it associates with fragmentation and disorganization of elastic fibers in several tissues, including the arterial wall. ATS is caused by pathogenic variants in SLC2A10 encoding the facilitative glucose transporter (GLUT)10. Critical Issues: Although several hypotheses have been forwarded, the molecular mechanisms linking disrupted GLUT10 activity with arterial malformations are largely unknown. Recent Advances: The vascular and systemic manifestations and natural history of ATS patients have been largely delineated. GLUT10 was identified as an intracellular transporter of dehydroascorbic acid, which contributes to collagen and elastin cross-linking in the endoplasmic reticulum, redox homeostasis in the mitochondria, and global and gene-specific methylation/hydroxymethylation affecting epigenetic regulation in the nucleus. We revise here the current knowledge on ATS and the role of GLUT10 within the compartmentalization of ascorbate in physiological and diseased states. Future Directions: Centralization of clinical, treatment, and outcome data will enable better management for ATS patients. Establishment of representative animal disease models could facilitate the study of pathomechanisms underlying ATS. This might be relevant for other forms of vascular dysplasia, such as isolated aneurysm formation, hypertensive vasculopathy, and neovascularization. Antioxid. Redox Signal . 34, 875-889.

Objective: Spontaneous cervicocerebral artery dissection (sCCD) is an important cause of ischaemic stroke that often occurs in young and middle aged patients. The purpose of this study was to investigate the correlation between tortuosity of the carotid artery and sCCD., Methods: Patients with confirmed sCCD who underwent computed tomography angiography (CTA) were reviewed retrospectively. Age and sex matched patients having CTA were used as controls. The tortuosity indices of the cervical arteries were measured from the CTA images. The carotid siphon and the extracranial internal carotid artery (ICA) were evaluated according to morphological classification. The carotid siphons were classified into five types. The extracranial ICA was categorised as simple tortuosity, coiling or kinking. Independent risk factors for sCCD were investigated using multivariable analysis., Results: The study included sixty-six patients with sCCD and 66 controls. There were no differences in vascular risk factors between the two groups. The internal carotid tortuosity index (ICTI) (25.24 ± 12.37 vs. 15.90 ± 8.55, respectively; p < .001) and vertebral tortuosity index (VTI) (median 11.28; interquartile range [IQR] 6.88, 18.80 vs. median 8.38; IQR 6.02, 12.20, respectively; p = .008) were higher in the patients with sCCD than in the controls. Type III and Type IV carotid siphons were more common in the patients with sCCD (p = .001 and p < .001, respectively). The prevalence of any vessel tortuosity, coiling and kinking of the extracranial ICA was higher in the patients with sCCD (p < .001, p = .018 and p = .006, respectively). ICTI (odds ratio [OR] 2.964; p = .026), VTI (OR 5.141; p = .009), and Type III carotid siphons (OR 4.654; p = .003) were independently associated with the risk of sCCD., Conclusion: Arterial tortuosity is associated with sCCD, and greater tortuosity of the cervical artery may indicate an increased risk of arterial dissection., (Copyright © 2020 European Society for Vascular Surgery. Published by Elsevier B.V. All rights reserved.)

Background: Vascular endothelial cells (ECs) are subject to continuous shear stress due to blood circulation. Mechanical stress due to high shear flow can also cause arteriovenous malformation (AVM) when ECs respond hyper-sensitively to shear flow. This study was conducted to test the hypothesis that angiogenesis could be promoted in response to mechanical stress via regulation of pro-angiogenic factors in AVM cells., Methods: ECs were extracted from the tissue samples from six AVM patients and six normal patients. Shear stress at 7 dynes/cm2 were applied for 24 h. Before and after application of shear stress to each group, RT-PCR was performed to access the expression levels of angiopoietin2(AGP2), aquaporin1(AQP1) and TGFβR1. Immunofluorescences was also performed to evaluate the level of protein expressions., Results: In both normal and AVM tissues, AGP2 and TGFβR1 under the shear stress showed increased expression in the ECs compared to the non-sheared samples. When AVMs and normal arterial vasculature were compared, the expression levels of both AGP2 and TGFβR1 in AVMs were higher when compared to normal arterial vasculature with or without shear stress. Immunofluorescence-based protein analysis also confirmed shear-induced AGP2 and TGFβR1 in both samples of normal and AVM patients., Conclusions: AVMs exhibited higher sensitivity to shear stress by producing higher expressions of some marked genes and proteins that regulate the endothelial functions upon exposure to shear stress. While the physiological mechanism for AVMs remain elusive, our study shows the plausibility of physical stress imposed by the shearing flow can cause the occurrence of AVMs.

Carpal tunnel syndrome (CTS) is the most common type of entrapment neuropathy. The majority of CTS cases are idiopathic and affect females between 40 and 60 years old. Conversely, this report describes two female patients in their mid-30's diagnosed with CTS caused by a median artery in the carpal tunnel using ultrasonography. We visualized the median artery which emerged from the radial artery and common interosseous artery in the proximal forearm of each patient by magnetic resonance angiography (MRA) before surgery. After the vertical incision of the transverse carpal ligament, the anomalous vessel was encountered, which ran over the median nerve at the radial aspect, and a simple mini-open procedure was performed for carpal tunnel release. Postoperatively, the CTS symptoms were relieved in both patients. The purpose of this report is to describe the persistent median artery using MRA in two patients and to report on their postoperative mini-open carpal tunnel release outcomes.