0 Midline1 Mid-lateral margin of the clivus or dorsum sellae2 Lateral-lateral margin of the clivus or dorsum sellae3 Cerebellopontine angle
Bifurcation height▲
0 Dorsum sellae or below1 Suprasellar cistern2 At the level of the third ventricle3 Recession and elevation of the floor of the third ventricle
AngioCT: computed tomography angiography; IADE: intracranial arterial dolichoectasia; mm: millimeters; * value of 1 is deemed abnormal; ▲ value of 2 or more suggests abnormality
Declarations
Author contributions
LDD, RRNRdN, and LSV: Conceptualization, Data curation, Formal analysis, Investigation, Methodology, Project administration, Resources, Software, Supervision, Validation, Visualization, Writing—original draft, Writing—review & editing. All authors have approved the final version of the manuscript and agree to be accountable for all aspects of the work, ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Conflicts of interest
The authors declare that they have no conflicts of interest.
Ethical approval
According to the International Committee of Medical Journal Editors (ICMJE) and Committee on Publication Ethics (COPE) guidelines, this review does not require ethical approval.
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The informed consent to participate from each participant has been obtained.
Consent to publication
The informed consent to publication was obtained from relevant participants.
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References
Pico F, Labreuche J, Amarenco P. Pathophysiology, presentation, prognosis, and management of intracranial arterial dolichoectasia.Lancet Neurol. 2015;14:833–45. [DOI] [PubMed]
Del Brutto VJ, Ortiz JG, Biller J. Intracranial Arterial Dolichoectasia.Front Neurol. 2017;8:344. [DOI] [PubMed] [PMC]
Ince B, Petty GW, Brown RD Jr, Chu CP, Sicks JD, Whisnant JP. Dolichoectasia of the intracranial arteries in patients with first ischemic stroke: A population-based study.Neurology. 1998;50:1694–8. [DOI] [PubMed]
Kobkitsuksakul C, Somboonnitiphol K, Apirakkan M, Lueangapapong P, Chanthanaphak E. Dolichoectasia of the internal carotid artery terminus, posterior communicating artery, and posterior cerebral artery: The embryonic caudal ramus internal carotid segmental vulnerability legacy.Interv Neuroradiol. 2020;26:124–30. [DOI] [PubMed] [PMC]
Zhai FF, Yan S, Li ML, Han F, Wang Q, Zhou LX, et al. Intracranial Arterial Dolichoectasia and Stenosis: Risk Factors and Relation to Cerebral Small Vessel Disease.Stroke. 2018;49:1135–40. [DOI] [PubMed]
Passero SG, Rossi S. Natural history of vertebrobasilar dolichoectasia.Neurology. 2008;70:66–72. [DOI] [PubMed]
GBD 2019 Stroke Collaborators. Global, regional, and national burden of stroke and its risk factors, 1990–2019: a systematic analysis for the Global Burden of Disease Study 2019.Lancet Neurol. 2021;20:795–820. [DOI] [PubMed] [PMC]
Bensenor IM, Goulart AC, Szwarcwald CL, Vieira ML, Malta DC, Lotufo PA. Prevalence of stroke and associated disability in Brazil: National Health Survey-2013.Arq Neuropsiquiatr. 2015;73:746–50. [DOI] [PubMed]
Brinjikji W, Nasr DM, Flemming KD, Rouchaud A, Cloft HJ, Lanzino G, et al. Clinical and Imaging Characteristics of Diffuse Intracranial Dolichoectasia.AJNR Am J Neuroradiol. 2017;38:915–22. [DOI] [PubMed] [PMC]
Nasr DM, Brinjikji W, Rouchaud A, Kadirvel R, Flemming KD, Kallmes DF. Imaging Characteristics of Growing and Ruptured Vertebrobasilar Non-Saccular and Dolichoectatic Aneurysms.Stroke. 2016;47:106–12. [DOI] [PubMed]
Chalouhi N, Ali MS, Jabbour PM, Tjoumakaris SI, Gonzalez LF, Rosenwasser RH, et al. Biology of Intracranial Aneurysms: Role of Inflammation.J Cereb Blood Flow Metab. 2012;32:1659–76. [DOI] [PubMed] [PMC]
Liu Q, Zhao B, Hu Q, Wang S, Zhu C. Editorial: Immunology and inflammation in intracranial aneurysms.Front Aging Neurosci. 2022;14:1046762. [DOI] [PubMed] [PMC]
Kassam AB, Horowitz M, Chang YF, Peters D. Altered arterial homeostasis and cerebral aneurysms: a molecular epidemiology study.Neurosurgery. 2004;54:1450–62. [DOI] [PubMed]
Owens GK. Molecular control of vascular smooth muscle cell differentiation.Acta Physiol Scand. 1998;164:623–35. [DOI] [PubMed]
Xu Y, Tian Y, Wei HJ, Chen J, Dong JF, Zacharek A, et al. Erythropoietin increases circulating endothelial progenitor cells and reduces the formation and progression of cerebral aneurysm in rats.Neuroscience. 2011;181:292–9. [DOI] [PubMed]
Tamura T, Jamous MA, Kitazato KT, Yagi K, Tada Y, Uno M, et al. Endothelial damage due to impaired nitric oxide bioavailability triggers cerebral aneurysm formation in female rats.J Hypertens. 2009;27:1284–92. [DOI] [PubMed]
Aoki T, Nishimura M, Matsuoka T, Yamamoto K, Furuyashiki T, Kataoka H, et al. PGE2-EP2 signalling in endothelium is activated by haemodynamic stress and induces cerebral aneurysm through an amplifying loop via NF-κB.Br J Pharmacol. 2011;163:1237–49. [DOI] [PubMed] [PMC]
Owens GK. Molecular control of vascular smooth muscle cell differentiation and phenotypic plasticity.Novartis Found Symp. 2007;283:174–93. [DOI] [PubMed]
Owens GK, Kumar MS, Wamhoff BR. Molecular Regulation of Vascular Smooth Muscle Cell Differentiation in Development and Disease.Physiol Rev. 2004;84:767–801. [DOI] [PubMed]
Yoshida T, Owens GK. Molecular Determinants of Vascular Smooth Muscle Cell Diversity.Circ Res. 2005;96:280–91. [DOI] [PubMed]
Aoki T, Nishimura M, Kataoka H, Ishibashi R, Nozaki K, Hashimoto N. Reactive oxygen species modulate growth of cerebral aneurysms: a study using the free radical scavenger edaravone and p47phox-/- mice.Lab Invest. 2009;89:730–41. [DOI] [PubMed]
Aoki T, Kataoka H, Nishimura M, Ishibashi R, Morishita R, Miyamoto S. Ets-1 promotes the progression of cerebral aneurysm by inducing the expression of MCP-1 in vascular smooth muscle cells.Gene Ther. 2010;17:1117–23. [DOI] [PubMed]
Kilic T, Sohrabifar M, Kurtkaya O, Yildirim O, Elmaci I, Günel M, et al. Expression of Structural Proteins and Angiogenic Factors in Normal Arterial and Unruptured and Ruptured Aneurysm Walls.Neurosurgery. 2005;57:997–1007. [DOI] [PubMed]
Austin G, Fisher S, Dickson D, Anderson D, Richardson S. The significance of the extracellular matrix in intracranial aneurysms.Ann Clin Lab Sci. 1993;23:97–105. [PubMed]
Loftus IM, Thompson MM. The role of matrix metalloproteinases in vascular disease.Vasc Med. 2002;7:117–33. [DOI] [PubMed]
Jones GT, Phillips VL, Harris EL, Rossaak JI, van Rij AM. Functional matrix metalloproteinase-9 polymorphism (C-1562T) associated with abdominal aortic aneurysm.J Vasc Surg. 2003;38:1363–7. [DOI] [PubMed]
Lamblin N, Bauters C, Hermant X, Lablanche JM, Helbecque N, Amouyel P. Polymorphisms in the promoter regions of MMP-2, MMP-3, MMP-9 and MMP-12 genes as determinants of aneurysmal coronary artery disease.J Am Coll Cardiol. 2002;40:43–8. [DOI] [PubMed]
Sho E, Sho M, Singh TM, Nanjo H, Komatsu M, Xu C, et al. Arterial Enlargement in Response to High Flow Requires Early Expression of Matrix Metalloproteinases to Degrade Extracellular Matrix.Exp Mol Pathol. 2002;73:142–53. [DOI] [PubMed]
Rabkin SW. The Role Matrix Metalloproteinases in the Production of Aortic Aneurysm.Prog Mol Biol Transl Sci. 2017;147:239–65. [DOI] [PubMed]
Jin D, Sheng J, Yang X, Gao B. Matrix metalloproteinases and tissue inhibitors of metalloproteinases expression in human cerebral ruptured and unruptured aneurysm.Surg Neurol. 2007;68:S16–7. [DOI] [PubMed]
Chyatte D, Bruno G, Desai S, Todor DR. Inflammation and Intracranial Aneurysms.Neurosurgery. 1999;45:1137–47. [DOI] [PubMed]
Frösen J, Piippo A, Paetau A, Kangasniemi M, Niemelä M, Hernesniemi J, et al. Remodeling of Saccular Cerebral Artery Aneurysm Wall Is Associated With Rupture: Histological Analysis of 24 Unruptured and 42 Ruptured Cases.Stroke. 2004;35:2287–93. [DOI] [PubMed]
Ishibashi R, Aoki T, Nishimura M, Hashimoto N, Miyamoto S. Contribution of Mast Cells to Cerebral Aneurysm Formation.Curr Neurovasc Res. 2010;7:113–24. [DOI] [PubMed]
Jayaraman T, Berenstein V, Li X, Mayer J, Silane M, Shin YS, et al. Tumor Necrosis Factor α is a Key Modulator of Inflammation in Cerebral Aneurysms.Neurosurgery. 2005;57:558–64. [DOI] [PubMed]
Polavarapu R, Gongora MC, Winkles JA, Yepes M. Tumor Necrosis Factor-Like Weak Inducer of Apoptosis Increases the Permeability of the Neurovascular Unit through Nuclear Factor-κB Pathway Activation.J Neurosci. 2005;25:10094–100. [DOI] [PubMed] [PMC]
Thomson EM, Williams A, Yauk CL, Vincent R. Overexpression of Tumor Necrosis Factor-α in the Lungs Alters Immune Response, Matrix Remodeling, and Repair and Maintenance Pathways.Am J Pathol. 2012;180:1413–30. [DOI] [PubMed]
Moriwaki T, Takagi Y, Sadamasa N, Aoki T, Nozaki K, Hashimoto N. Impaired Progression of Cerebral Aneurysms in Interleukin-1β-Deficient Mice.Stroke. 2006;37:900–5. [DOI] [PubMed]
Ait-Oufella H, Taleb S, Mallat Z, Tedgui A. Recent Advances on the Role of Cytokines in Atherosclerosis.Arterioscler Thromb Vasc Biol. 2011;31:969–79. [DOI] [PubMed]
Jayaraman T, Paget A, Shin YS, Li X, Mayer J, Chaudhry H, et al. TNF-α-mediated inflammation in cerebral aneurysms: A potential link to growth and rupture.Vasc Health Risk Manag. 2008;4:805–17. [DOI] [PubMed] [PMC]
Sprague AH, Khalil RA. Inflammatory cytokines in vascular dysfunction and vascular disease.Biochem Pharmacol. 2009;78:539–52. [DOI] [PubMed] [PMC]
Tedgui A, Mallat Z. Cytokines in Atherosclerosis: Pathogenic and Regulatory Pathways.Physiol Rev. 2006;86:515–81. [DOI] [PubMed]
Tulamo R, Frösen J, Junnikkala S, Paetau A, Pitkäniemi J, Kangasniemi M, et al. Complement activation associates with saccular cerebral artery aneurysm wall degeneration and rupture.Neurosurgery. 2006;59:1069–77. [DOI] [PubMed]
Tulamo R, Frösen J, Junnikkala S, Paetau A, Kangasniemi M, Peláez J, et al. Complement system becomes activated by the classical pathway in intracranial aneurysm walls.Lab Invest. 2010;90:168–79. [DOI] [PubMed]
Jia ZY, Zhao LB, Lee DH. Localized Marked Elongation of the Distal Internal Carotid Artery with or without PHACE Syndrome: Segmental Dolichoectasia of the Distal Internal Carotid Artery.AJNR Am J Neuroradiol. 2018;39:817–23. [DOI] [PubMed] [PMC]
Zhang DP, Yin S, Zhang HL, Li D, Song B, Liang JX. Association between Intracranial Arterial Dolichoectasia and Cerebral Small Vessel Disease and Its Underlying Mechanisms.J Stroke. 2020;22:173–84. [DOI] [PubMed] [PMC]
Kurtelius A, Väntti N, Rezai Jahromi B, Tähtinen O, Manninen H, Koskenvuo J, et al. Association of Intracranial Aneurysms With Aortic Aneurysms in 125 Patients With Fusiform and 4253 Patients With Saccular Intracranial Aneurysms and Their Family Members and Population Controls.J Am Heart Assoc. 2019;8:e013277. [DOI] [PubMed] [PMC]
Flemming KD, Wiebers DO, Brown RD Jr, Link MJ, Huston J 3rd, McClelland RL, et al. The Natural History of Radiographically Defined Vertebrobasilar Nonsaccular Intracranial Aneurysms.Cerebrovasc Dis. 2005;20:270–9. [DOI] [PubMed]
Chen GC, Neelakantan N, Martín-Calvo N, Koh WP, Yuan JM, Bonaccio M, et al. Adherence to the Mediterranean diet and risk of stroke and stroke subtypes.Eur J Epidemiol. 2019;34:337–49. [DOI] [PubMed]
Bliss ES, Wong RHX, Howe PRC, Mills DE. The Effects of Aerobic Exercise Training on Cerebrovascular and Cognitive Function in Sedentary, Obese, Older Adults.Front Aging Neurosci. 2022;14:892343. [DOI] [PubMed] [PMC]
Shapiro M, Becske T, Riina HA, Raz E, Zumofen D, Nelson PK. Non-saccular vertebrobasilar aneurysms and dolichoectasia: a systematic literature review.J Neurointerv Surg. 2014;6:389–93. [DOI] [PubMed]
Smoker WR, Price MJ, Keyes WD, Corbett JJ, Gentry LR. High-resolution computed tomography of the basilar artery: 1. Normal size and position.AJNR Am J Neuroradiol. 1986;7:55–60. [PubMed] [PMC]
Hassan T, Ezura M, Timofeev EV, Tominaga T, Saito T, Takahashi A, et al. Computational simulation of therapeutic parent artery occlusion to treat giant vertebrobasilar aneurysm.AJNR Am J Neuroradiol. 2004;25:63–8. [PubMed] [PMC]
Brinjikji W, Chung B, Yong-Hong D, Wald JT, Mut F, Kadirvel R, et al. Hemodynamic characteristics of stable and unstable vertebrobasilar dolichoectatic and fusiform aneurysms.J Neurointerv Surg. 2018;10:1102–7. [DOI] [PubMed]
Zhang H, Liang S, Lv X. Intra-aneurysmal thrombosis and turbulent flow on MRI of large and giant internal carotid artery aneurysms.Neurosci Inform. 2021;1:100027. [DOI]
Lehman VT, Brinjikji W. Vessel Wall Imaging of Unruptured Intracranial Aneurysms: Ready for Prime Time?AJNR Am J Neuroradiol. 2019;40:E26–9. [DOI] [PubMed] [PMC]
Larsen N, von der Brelie C, Trick D, Riedel CH, Lindner T, Madjidyar J, et al. Vessel Wall Enhancement in Unruptured Intracranial Aneurysms: An Indicator for Higher Risk of Rupture? High-Resolution MR Imaging and Correlated Histologic Findings.AJNR Am J Neuroradiol. 2018;39:1617–21. [DOI] [PubMed] [PMC]
Germain DP, Altarescu G, Barriales-Villa R, Mignani R, Pawlaczyk K, Pieruzzi F, et al. An expert consensus on practical clinical recommendations and guidance for patients with classic Fabry disease.Mol Genet Metab. 2022;137:49–61. [DOI] [PubMed]
Wilcox WR, Oliveira JP, Hopkin RJ, Ortiz A, Banikazemi M, Feldt-Rasmussen U, et al. Females with Fabry disease frequently have major organ involvement: lessons from the Fabry Registry.Mol Genet Metab. 2008;93:112–28. [DOI] [PubMed]
Politei J, Schenone AB, Burlina A, Blanco M, Lescano S, Szlago M, et al. Vertebrobasilar Dolichoectasia in Fabry Disease.J Inborn Errors Metab Screen. 2014;2:1–6. [DOI]
Desnick RJ, Brady R, Barranger J, Collins AJ, Germain DP, Goldman M, et al. Fabry Disease, an Under-Recognized Multisystemic Disorder: Expert Recommendations for Diagnosis, Management, and Enzyme Replacement Therapy.Ann Intern Med. 2003;138:338–46. [DOI] [PubMed]
Toyoshima Y, Emura I, Umeda Y, Fujita N, Kakita A, Takahashi H. Vertebral basilar system dolichoectasia with marked infiltration of IgG4-containing plasma cells: A manifestation of IgG4-related disease?Neuropathology. 2012;32:100–4. [DOI] [PubMed]
Saitakis G, Chwalisz BK. The neurology of IGG4-related disease.J Neurol Sci. 2021;424:117420. [DOI] [PubMed]
Wallace ZS, Naden RP, Chari S, Choi H, Della-Torre E, Dicaire JF, et al.; American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working Group. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease.Arthritis Rheumatol. 2020;72:7–19. [DOI] [PubMed]
Kim ST, Brinjikji W, Lanzino G, Kallmes DF. Neurovascular manifestations of connective-tissue diseases: A review.Interv Neuroradiol. 2016;22:624–37. [DOI] [PubMed] [PMC]
Schievink WI, Torres VE, Wiebers DO, Huston J 3rd. Intracranial arterial dolichoectasia in autosomal dominant polycystic kidney disease.J Am Soc Nephrol. 1997;8:1298–303. [DOI] [PubMed]
Johnston SC, Amarenco P, Denison H, Evans SR, Himmelmann A, James S, et al.; THALES Investigators. Ticagrelor and Aspirin or Aspirin Alone in Acute Ischemic Stroke or TIA.N Engl J Med. 2020;383:207–17. [DOI] [PubMed]
Toyoda K, Uchiyama S, Yamaguchi T, Easton JD, Kimura K, Hoshino H, et al.; CSPS.com Trial Investigators. Dual antiplatelet therapy using cilostazol for secondary prevention in patients with high-risk ischaemic stroke in Japan: a multicentre, open-label, randomised controlled trial.Lancet Neurol. 2019;18:539–48. [DOI] [PubMed]
Shinohara Y, Katayama Y, Uchiyama S, Yamaguchi T, Handa S, Matsuoka K, et al.; CSPS 2 group. Cilostazol for prevention of secondary stroke (CSPS 2): an aspirin-controlled, double-blind, randomised non-inferiority trial.Lancet Neurol. 2010;9:959–68. [DOI] [PubMed]
Greving JP, Wermer MJ, Brown RD Jr, Morita A, Juvela S, Yonekura M, et al. Development of the PHASES score for prediction of risk of rupture of intracranial aneurysms: a pooled analysis of six prospective cohort studies.Lancet Neurol. 2014;13:59–66. [DOI] [PubMed]
Bijlenga P, Gondar R, Schilling S, Morel S, Hirsch S, Cuony J, et al. PHASES Score for the Management of Intracranial Aneurysm: A Cross-Sectional Population-Based Retrospective Study.Stroke. 2017;48:2105–112. [DOI] [PubMed]
Backes D, Vergouwen MD, Tiel Groenestege AT, Bor AS, Velthuis BK, Greving JP, et al. PHASES Score for Prediction of Intracranial Aneurysm Growth.Stroke. 2015;46:1221–6. [DOI] [PubMed]
Jung YJ, Kim MS, Choi BY, Chang CH. Fusiform Aneurysm on the Basilar Artery Trunk Treated with Intra-Aneurysmal Embolization with Parent Vessel Occlusion after Complete Preoperative Occlusion Test.J Korean Neurosurg Soc. 2013;53:235–40. [DOI] [PubMed] [PMC]
Siddiqui AH, Abla AA, Kan P, Dumont TM, Jahshan S, Britz GW, et al. Panacea or problem: flow diverters in the treatment of symptomatic large or giant fusiform vertebrobasilar aneurysms.J Neurosurg. 2012;116:1258–66. [DOI] [PubMed]
Little JR, St Louis P, Weinstein M, Dohn DF. Giant fusiform aneurysm of the cerebral arteries.Stroke. 1981;12:183–8. [DOI] [PubMed]
Kim SM, Jung JM, Kim BJ, Lee JS, Kwon SU. Cilostazol Mono and Combination Treatments in Ischemic Stroke: An Updated Systematic Review and Meta-Analysis.Stroke. 2019;50:3503–11. [DOI] [PubMed]
Giannantoni NM, Broccolini A, Frisullo G, Pilato F, Profice P, Morosetti R, et al. Neurofibromatosis Type 1 Associated with Vertebrobasilar Dolichoectasia and Pontine Ischemic Stroke.J Neuroimaging. 2015;25:505–6. [DOI] [PubMed]
Torres VE, Harris PC. Autosomal dominant polycystic kidney disease: the last 3 years.Kidney Int. 2009;76:149–68. [DOI] [PubMed] [PMC]
Byers PH, Murray ML. Heritable Collagen Disorders: The Paradigm of the Ehlers—Danlos Syndrome.J Invest Dermatol. 2012;132:E6–11. [DOI] [PubMed]
Lv X, Yu J, Liao T, Wang J, Wang G. Unruptured giant intracavernous aneurysms untolerate internal carotid artery occlusion test: Untreated and treated with flow-diversion.Neuroradiol J. 2020;33:105–11. [DOI] [PubMed] [PMC]
Lawton MT, Abla AA, Rutledge WC, Benet A, Zador Z, Rayz VL, et al. Bypass Surgery for the Treatment of Dolichoectatic Basilar Trunk Aneurysms: A Work in Progress.Neurosurgery. 2016;79:83–99. [DOI] [PubMed] [PMC]
