Multimodality Imaging of Vertebrobasilar Dolichoectasia: Clinical Presentations and Imaging Spectrum

Abstract

Vertebrobasilar dolichoectasia (VBD) is characterized by ectasia, elongation, and tortuosity of the vertebrobasilar arteries, with a high degree of variability in clinical presentation. The disease origin is believed to involve degeneration of the internal elastic lamina, thinning of the media secondary to reticular fiber deficiency, and smooth muscle atrophy. The prevalence of VBD is variable, ranging from 0.05% to 18%. Most patients with VBD are asymptomatic and their VBD is detected incidentally; however, it is important to recognize that the presence of symptoms, which can lead to clinically significant morbidity and sometimes mortality, may influence clinical management. The most important clinical presentations of VBD are vascular events, such as ischemic stroke and catastrophic intracranial hemorrhage, or progressive compressive symptoms related to compression of adjacent structures, including the cranial nerves, brainstem, or third ventricle, causing hydrocephalus. The imaging diagnostic criteria for computed tomography and magnetic resonance (MR) imaging include three quantitative measures of basilar artery morphology: laterality score, height of bifurcation, and basilar artery diameter. The authors review the relevant anatomy and disease origin of VBD; pertinent imaging findings, including intraluminal thrombus and relation to the cranial nerves; and imaging pitfalls, such as the hyperintense vessel sign on MR images and artifacts related to slow flow in the dolichoectatic vessel. In addition, clinical manifestations, the role of radiology in diagnosis and management of this condition, and available management options are reviewed. (©)RSNA, 2016.

Deletion of 6p25.3 Is Associated with Cerebrovascular Dolichoectasia: Report of 2 Cases

“…FOXF2 forms a gene tandem triplet with FOXC1 and FOXQ1 at 6p25.3 and concurrent deletion of these genes results from 6p25.3 chromosomal deletions [4]. Intracranial dolichoectasia is most commonly expressed in the posterior circulation, predominantly in the vertebrobasilar system [7, 9]. Depending on the level of deviation of the basilar artery, compression of cranial nerves may occur, particularly the trigeminal and facial nerves, causing facial muscle spasm and diminished sensation in the face.…”

Perfusion by delayed time to peak in vertebrobasilar dolichoectasia patients with vertigo

“…Vertebrobasilar dolichoectasia (VBD) was a clinical entity characterized by elongated, dilated and/or tortuous vertebral artery, and basilar artery (BA),1, 2, 3 ranging from 0.05% to 18% 4, 5, 6. VBD presentation includes symptoms by cerebral ischemia in the vertebrobasilar territory 2, 3, 4, 5, 6…”

“…VBD presentation includes symptoms by cerebral ischemia in the vertebrobasilar territory 2, 3, 4, 5, 6…”

Ultrasound Findings Disclose the Mutual Impact of Vertebrobasilar Dolichoectasia and Vertebral Artery Hypoplasia

“…Vertebrobasilar dolichoectasia (VBD), a vasculopathy characterized by structural features of elongation, tortuosity, and dilatation of the vertebral artery (VA) with or without basilar artery (BA) involvement, is mostly commonly identified by brain magnetic resonance imaging . The prevalence has differed among the general population, ranging from 0.05% to 18% according to different studied populations; the clinical symptoms have also been reported to vary from asymptomatic to focal neurologic signs related to the compression of nearby structures or ischemia strokes …”

“…V ertebrobasilar dolichoectasia (VBD), a vasculopathy characterized by structural features of elongation, tortuosity, and dilatation of the vertebral artery (VA) with or without basilar artery (BA) involvement, 1-3 is mostly commonly identified by brain magnetic resonance imaging. 1,[4][5][6] The prevalence has differed among the general population, ranging from 0.05% to 18% according to different studied populations 1,2,7,8 ; the clinical symptoms have also been reported to vary from asymptomatic to focal neurologic signs related to the compression of nearby structures or ischemia strokes. 6,[9][10][11][12][13] In contrast to VBD, vertebral artery hypoplasia (VAH) is characterized by small-caliber or reduced flow volume in the affected VA and is either diagnosed by carotid artery ultrasound (US) of the diameter or blood flow volume measurements and brain imaging.…”