Abstract 16: Lenticulostriate Collaterals Predict Angiographic Course in Unilateral Focal Cerebral Arteriopathy of Childhood
Introduction: Focal cerebral arteriopathy (FCA) is a frequent cause of stroke in children and an important risk factor for stroke recurrence. The factors affecting progression of FCA remain undetermined.
Hypothesis: We hypothesized that the presence of lenticulostriate (LS) collaterals at presentation can serve as a prognostic marker of progressive arteriopathy in children with unilateral FCA.
Methods: Children aged 1-month to 18-years with FCA, defined as unilateral steno-occlusive disease at the carotid terminus, were retrospectively identified from two centers. Presence of collaterals was based on digital subtraction angiography (DSA) at baseline. The patients’ imaging (MR angiogram or DSA) dataset at baseline and last follow-up was reviewed by 2 blinded raters and their outcomes classified as improved, stable, or progressed based on location and degree of steno-occlusive disease. The presence or absence of LS collaterals at presentation and angiographic outcomes were compared using Fisher’s exact test.
Results: Our study included 46 children; 24 males, with median age 8.2 years (range 2-17.4 years), of which 21 had LS collaterals at presentation (46%). Median follow-up periods were 12 months and 19 months for the collateral and non-collateral groups, respectively. Angiographic improvement occurred exclusively in the non-collateral group (48%) (p=0.0001). In the collateral group, progression occurred ipsilaterally in 29%, and contralaterally in 14%. In the non-collateral group, progression was only ipsilateral, occurring in 35%. Discordant progression, defined as progression and improvement across multiple vessels, only occurred in the non-collateral group (67%) (p=0.02). No patients in the non-collateral group developed LS collaterals.
Conclusion: The presence of LS collaterals in FCA was associated with ipsilateral or contralateral progression, while their absence was associated with ipsilateral progression or improvement. We conclude that LS collaterals in FCA likely signify a chronic, progressive process recognized as Moyamoya disease. In the absence of collaterals, steno-occlusion may progress in a discordant fashion or improve, indicating a varied vascular response to injury or inflammation, and the capacity to heal.
Author Disclosures: J. Elbers: None. S.M. Benseler: None. D. Armstrong: None. G.K. Steinberg: None. K.W. Yeom: None.
- © 2015 by American Heart Association, Inc.