Introduction: Childhood cerebral arteriopathies are heterogeneous and difficult to classify. Our goal was to identify clinical and imaging biomarkers associated with subtypes of childhood arteriopathy in order to facilitate their diagnosis and classification in both research and clinical settings.
Methods: From 2009-2014, the VIPS study enrolled 355 children (age 29d-18y) with arterial ischemic stroke. A neuroradiologist and pediatric vascular neurologist independently reviewed vascular imaging and clinical data to diagnose arteriopathy (none, possible, definite), and then classify arteriopathy subtype: arterial dissection, transient cerebral arteriopathy (TCA), moyamoya, and secondary vasculitis. Disagreements were resolved through discussion by a panel of two neuroradiologists and two pediatric vascular neurologists. We constructed multivariable logistic regression models to identify characteristics independently associated with each subtype.
Results: Of 127 cases with definite arteriopathy, 109 received a single arteriopathy subtype diagnosis (26 dissection, 25 TCA, 34 moyamoya, 15 secondary vasculitis, and 9 “other”), while 18 were of indeterminate subtype. There were no cases of primary vasculitis. Independent predictors of arteriopathy subtype are shown (Table). The association between black race and moyamoya is explained by sickle cell anemia. A banding pattern on the vascular imaging was pathognomonic of TCA. Moyamoya and vasculitis secondary to meningitis had similar distal internal carotid artery abnormalities, but lenticulostriate collaterals suggested moyamoya, and a decreased level of consciousness predicted secondary vasculitis.
Conclusions: The different subtypes of childhood arteriopathies are associated with typical clinical, parenchymal and vascular imaging features that can help narrow the differential diagnosis in pediatric stroke patients with vascular abnormalities.