Arterial Tortuosity: An Imaging Biomarker of Childhood Stroke Pathogenesis?

Abstract

Background and Purpose Arteriopathy is the leading cause of childhood arterial ischemic stroke (AIS). Mechanisms are poorly understood but may include inherent abnormalities of arterial structure. Extracranial dissection is associated with connective tissue disorders in adult stroke. Focal cerebral arteriopathy (FCA) is a common syndrome where pathophysiology is unknown but may include intracranial dissection or transient cerebral arteriopathy (TCA). We aimed to quantify cerebral arterial tortuosity in childhood AIS, hypothesizing increased tortuosity in dissection. Methods Children (1month-18 years) with AIS were recruited within the Vascular Effects of Infection in Pediatric Stroke (VIPS) study with controls from the Calgary Pediatric Stroke Program. Objective, multi-investigator review defined diagnostic categories. A validated imaging software method calculated mean arterial tortuosity of the major cerebral arteries using 3D time-of-flight MR angiography source images. Tortuosity of unaffected vessels was compared between children with dissection, TCA, meningitis, moyamoya, cardioembolic strokes and controls (ANOVA, post-hoc Tukey). Trauma-related versus spontaneous dissection was compared (Student's t-test). Results One hundred fifteen children were studied (median 6.8 years, 43% female). Age and sex were similar across groups. Tortuosity means and variances were consistent with validation studies. Tortuosity in controls (1.346±0.074,n=15) was comparable to moyamoya (1.324±0.038, n=15, p=0.998), meningitis (1.348±0.052, n=11, p=0.989) and cardioembolic (1.379±0.056, n=27, p=0.190) cases. Tortuosity was higher in both extracranial dissection (1.404±0.084, n=22, p=0.021) and TCA (1.390±0.040, n=27, p=0.001) children. Tortuosity was not different between traumatic versus spontaneous dissection (p=0.70). Conclusion In children with dissection and TCA cerebral arteries demonstrate increased tortuosity. Quantified arterial tortuosity may represent a clinically relevant imaging biomarker of vascular biology in pediatric stroke.

Incidence, kinetics, and risk factors for intra- and extracranial cerebral arteriopathies in a newborn sickle cell disease cohort early assessed by transcranial and cervical color Doppler ultrasound

“…Kinkings are known to be increased in a variety of connective tissue genetic disorders, such as Loeys–Dietz syndrome ( 29 ). They are associated with a risk of eICA dissection ( 30 ) and transient cerebral arteriopathy and may represent a clinically relevant imaging biomarker of vascular biology for pediatric strokes ( 31 ). The congenital or acquired origin of kinkings is still debated ( 32 ).…”

Quantitative Assessment of the Intracranial Vasculature of Infants and Adults Using iCafe (Intracranial Artery Feature Extraction)

“…In adults, the value of automated quantitative vascular imaging measures to investigate central nervous system diseases has been demonstrated (4,16). Though less commonly applied in (26,27). In those studies, the quantitative approach utilized was limited to artery tortuosity measurements of major arterial branches without reproducibility assessments.…”

Clinical variability in two Macedonian families with Arterial tortuosity syndrome

“…[ 13 ] of carotid artery, vertebral artery, and vertebro-basilar junction tortuosity on MRA imaging among patients with recurrent headache further supports the idea that tortuosity of the arteries responsible for brain perfusion may lead to migraine/headache. Furthermore, arterial tortuosity has been shown to be a risk factor for cerebral ischemic events and extracerebral dissection of arteries [ 14 ]. Moreover, several patients with ATS and stroke have been reported [ 1 , 15 ].…”