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. 2025 Jun;5(2):100265.
doi: 10.1016/j.ynirp.2025.100265. Epub 2025 May 9.

Cerebral arterial lumens are enlarged in children and young adults with sickle cell disease compared to peers

Josiah B Lewis  1   2 , Melanie E Fields  1   2 , Michael M Binkley  1 , Anita Zhou  1 , Amy Mirro  1   3 , Amy Ouyang  1   2 , Niket Gupta  3 , Yasheng Chen  1 , Slim Fellah  1 , Alyssa E Smith  1   2 , Igor Dedkov  1   2 , Monica L Hulbert  2 , Andria L Ford  1   3 , Hongyu An  1   3 , Jin-Moo Lee  1   3 , Manu S Goyal  3 , Kristin P Guilliams  1   2   3
Affiliations

Cerebral arterial lumens are enlarged in children and young adults with sickle cell disease compared to peers

Josiah B Lewis et al. Neuroimage Rep. 2025 Jun.

Abstract

Children with sickle cell disease (SCD) may develop large vessel narrowing, but studies suggest vessels may also be enlarged, possibly related to increased cerebral blood flow (CBF). We used MRI to investigate whether the cross-sectional total inflow vessel luminal area (TIVLA) proximal to the circle of Willis (carotid arteries + basilar artery) would be increased in SCD compared to age- and sex-matched peers after adjusting for CBF. Across 36 children with SCD (19 female, median age 10.7 [8.0-14.5] years and 43 controls (26 female, median age 12.7 [9.2-18.2] years) matched by age (p = 0.13) and sex (p = 0.50), the median TIVLA in the SCD group (35.9 mm2 [30.7, 39.5]) was larger than controls (30.5 mm2 [27.8, 35.4], p = 0.002). In a mixed model including age, sex, hemoglobin, CBF, SCD status, and an interaction between hemoglobin and SCD status, CBF (β = 0.11, CI 0.02-0.20, p = 0.02), SCD (β = 28.02, CI 5.62-50.42, p = 0.015), and the interaction between SCD and hemoglobin (β = -2.48, CI -4.49 to -0.47, p = 0.018) were all significantly associated with increased TIVLA. Notably, TIVLA as a measure of arterial lumens is larger in children with SCD, even after adjusting for CBF in the mixed model. This implies disease-specific normative values may be needed to detect early vasculopathy.

Keywords: Arterial spin labeling; Brain development; Cerebral blood flow; Cerebrovascular disease; Hematology; MR angiography; MRI.

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Conflict of interest statement

CRediT authorship contribution statement Josiah B. Lewis: Writing – review & editing, Writing – original draft, Visualization, Validation, Software, Methodology, Investigation, Formal analysis. Melanie E. Fields: Writing – review & editing, Writing – original draft, Investigation. Michael M. Binkley: Writing – review & editing, Formal analysis. Anita Zhou: Writing – review & editing, Validation, Investigation, Formal analysis. Amy Mirro: Writing – review & editing, Software, Investigation. Amy Ouyang: Writing – review & editing, Investigation. Niket Gupta: Writing – review & editing, Methodology, Investigation. Yasheng Chen: Writing – review & editing, Software, Methodology. Slim Fellah: Writing – review & editing, Software, Investigation. Alyssa E. Smith: Writing – review & editing, Investigation. Igor Dedkov: Writing – review & editing, Visualization, Investigation. Monica L. Hulbert: Writing – review & editing, Investigation. Andria L. Ford: Writing – review & editing, Investigation. Hongyu An: Writing – review & editing, Investigation. Jin-Moo Lee: Writing – review & editing, Conceptualization. Manu S. Goyal: Writing – review & editing, Writing – original draft, Methodology, Investigation, Conceptualization. Kristin P. Guilliams: Writing – review & editing, Writing – original draft, Supervision, Project administration, Methodology, Investigation, Conceptualization.

Figures

Image 1
Graphical abstract
Fig. 1
Fig. 1
Comparison of distal basilar cross-sectional area between representative subjects: both 11-year-old females, one with SCD (top), the other a control (bottom). TOF-MRA axial slices (left) and zoomed basilar region (right). The area of the distal basilar segment for the SCD participant is ∼7 % higher, and the TIVLA is ∼20 % higher.
Fig. 2
Fig. 2
Density ‘violin’ plot showing TIVLA from SCD and control cohorts. Median values are represented by coarsely dashed lines, interquartile ranges by finely dashed lines.
Fig. 3
Fig. 3
Univariate correlations with Pearson correlation coefficients (ρ), significance (p), and linear regression (R2) showing (a) age, (b) whole brain cerebral blood flow, and (c) hemoglobin against TIVLA. Lesion volume is also plotted against TIVLA, along with a fit to the probability of having a lesion volume of any size detected, with Wilcoxon Z (d). 95 % confidence intervals are plotted in blue.
See this image and copyright information in PMC

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