Clinical and Imaging Characteristics of Arteriopathy Subtypes in Children with Arterial Ischemic Stroke: Results of the VIPS Study

Abstract

Introduction We quantified clinical and imaging characteristics associated with childhood arteriopathy subtypes to facilitate their diagnosis and classification in research and clinical settings. Methods The “Vascular effects of Infection in Pediatric Stroke” (VIPS) study prospectively enrolled 355 children with arterial ischemic stroke (AIS) (2010–2014). A central team of experts reviewed all data to diagnose childhood arteriopathy and classify subtypes, including arterial dissection, focal cerebral arteriopathy-inflammatory type (FCA-i, which includes transient cerebral arteriopathy, TCA), moyamoya, and diffuse/multifocal vasculitis. Only children whose stroke etiology could be conclusively diagnosed were included in these analyses. We constructed logistic regression models to identify characteristics associated with each arteriopathy subtype. Results Among 127 children with definite arteriopathy, the arteriopathy subtype could not be classified in 18 (14%). Moyamoya (n=34) occurred mostly in children <8 years old, FCA-i (n=25) in 8–15 year olds, and dissection (n=26) at all ages. Vertigo at stroke presentation was common in dissection. Dissection affected cervical arteries, while moyamoya involved supraclinoid internal carotid arteries. A banded appearance of the M1 segment of the middle cerebral artery was pathognomonic of FCA-i, but present in <25% of FCA-i cases; a small lenticulostriate distribution infarct was a more common predictor of FCA-i, present in 76%. It remained difficult to distinguish FCA-i from intracranial dissection of the anterior circulation (FCA-d). We observed only secondary forms of diffuse/multifocal vasculitis, mostly due to meningitis. Conclusions Childhood arteriopathy subtypes have some typical features that aid diagnosis. Better imaging methods, including vessel wall imaging, are needed for improved classification of FCA.

Neuroimaging of Pediatric Arteriopathies

“…Abrupt occlusion without arterial wall anomalies is not sufficient to diagnose arteriopathy, as it is typically seen in embolic events . The most commonly reported presentation of arteriopathies in children is acute arterial ischemic stroke (AIS) . Arteriopathies represent a strong predictor of recurrent stroke; recurrent rates are reported to be >30% within 12 months, highest for Moyamoya syndrome (MMS), followed by focal cerebral arteriopathy (FCA) and dissection …”

“…Based on anatomic features, previously used term such as transient cerebral arteriopathy (TCA), FCA of childhood, large‐vessel childhood primary angitis of the central nervous system (cPACNS), and steno‐occlusive arteriopathy are now unified into unilateral FCA of childhood. Similarly, Moyamoya and fibromuscular dysplasia (FMD) are now included in the bilateral cerebral arteriopathies of childhood . Although we will be using some of old terminologies, we will also mention the corresponding CASCADE criteria definition wherever possible.…”

“…Also, we have some suggestions for the CASCADE criteria of definite SVA of childhood and their secondary classification of AIS. It should be noted that with the evolving knowledge of pathophysiologies, definitions of arteriopathies are being regularly updated …”

“…As the clinical and neuroimaging features and course of nonprogressive cPACNS are similar to other childhood inflammatory vasculitis such as TCA, post‐varicella angiopathy and FCA of childhood, the CASCADE criteria replaced these overlapping terminologies. These are now unified into one classification as unilateral focal FCA of the anterior circulation without collaterals, described above . The CASCADE criteria merged angiography positive, progressive cPACNS into unilateral focal FCA of anterior circulation with collaterals .…”

“…1 The most commonly reported presentation of arteriopathies in children is acute arterial ischemic stroke (AIS). 2,3 Arteriopathies represent a strong predictor of recurrent stroke; recurrent rates are reported to be >30% within 12 months, highest for Moyamoya syndrome (MMS), followed by focal cerebral arteriopathy (FCA) and dissection. [4][5][6][7] Although vascular occlusive disorders are more likely to cause AIS, this is not always true.…”