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Arterial spin-labeled (ASL) perfusion in children with Sturge-Weber syndrome: a retrospective cross-sectional study

  • Paediatric Neuroradiology
  • Published:
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Abstract

Purpose

Sturge-Weber syndrome (SWS) is a developmental disorder with venous hypertension and associated tissue responses including pial angiomatosis, cortical calcifications, and cerebral atrophy. Arterial spin-labeled (ASL) perfusion is an advanced MR sequence which can assess perfusion, without the need for contrast. We systematically evaluated the potential benefits of using ASL in Sturge-Weber syndrome, to determine the extent of intracranial perfusion abnormality and stage of disease, relevant for prognostication and surgical planning.

Methods

Two pediatric neuroradiologists retrospectively evaluated ASL perfusion imaging of 31 children with confirmed SWS and recorded the presence of hyper-perfusion, hypo-perfusion, or normal perfusion. The presence and distribution of ASL abnormality were compared against the presence and side of atrophy/calcification and pial angiomatosis on standard MR sequences.

Results

Thirty-one children (52% female, median age 16.7 months) with SWS had ASL imaging. Seven (23%) had hyper-perfusion, 15 (48%) had hypo-perfusion, and 9 (29%) had no perfusion abnormalities. ASL perfusion abnormality matched the location of SWS findings on conventional imaging in 86% (19/22). ASL demonstrated statistically significant increased perfusion in the early stage of the disease and decreased perfusion when there was atrophy. The parietal lobe was involved in 86% of cases.

Conclusion

ASL perfusion imaging is an advanced technique which may contribute to earlier diagnosis and more accurate prognostication of Sturge-Weber syndrome, helping guide management and potential surgical planning.

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Data Availability

The raw data supporting the findings of this study are not publicly available to preserve individuals’ privacy, although are available from the corresponding author upon reasonable request.

Abbreviations

ASL:

Arterial spin labeled

SWI:

Susceptibility weighted imaging

MR:

Magnetic resonance

SWS:

Sturge-Weber syndrome

SPECT:

Single-photon emission tomography

IQR:

Inter quartile range

PWI:

Perfusion weighted imaging

BOLD:

Blood oxygen level–dependent

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Funding

The authors did not receive support from any organization for the submitted work.

No funding was received to assist with the preparation of this manuscript.

No funding was received for conducting this study.

No funds, grants, or other support was received.

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Authors and Affiliations

  1. Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Simon M. Clifford, Adarsh Ghosh, Alireza Zandifar, Luis O. Tierradentro-García, Jorge D. U. Kim & Savvas Andronikou

  2. Department of Radiology, Children’s Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA

    Simon M. Clifford & Savvas Andronikou

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  1. Simon M. Clifford
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Correspondence to Simon M. Clifford.

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Ethical approval

Ethical approval was waived by the local Ethics Committee of Children’s Hospital of Philadelphia, in view of the retrospective nature of the study and all the procedures being performed were part of the routine care (Children’s Hospital of Philadelphia Institutional Review Board protocol ID: IRB 20–017336).

Informed consent

The Children’s Hospital of Philadelphia institutional review board approved this retrospective observational study. The need for informed consent was waived, with all the imaging having been performed as part of routine care, and there was no concern about identifying information (Children’s Hospital of Philadelphia Institutional Review Board protocol ID: IRB 20–017336).

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Clifford, S.M., Ghosh, A., Zandifar, A. et al. Arterial spin-labeled (ASL) perfusion in children with Sturge-Weber syndrome: a retrospective cross-sectional study. Neuroradiology 65, 1825–1834 (2023). https://doi.org/10.1007/s00234-023-03224-8

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