Neuropediatrics 2024; 55(S 01): S1-S25
DOI: 10.1055/s-0044-1791893
Durchblutungsstörungen des ZNS

Volumetric Analysis of Brain Volumes in Pediatric Moyamoya Patients before Revascularization Surgery

Authors

  • G. Oesch

    1   Inselspital, Universitätsspital Bern, Universitätsklinik für Kinderheilkunde, Neuropädiatrie, Entwicklung und Rehabilitation, Bern, Switzerland

  • R. O'Gorman Tuura

    3   Kinderuniversitätsspital Zürich, Zentrum für MR- Forschung, Zürich, Switzerland

  • M. Hebeisen

    4   Universität Zürich, Biostatistik, Zürich, Switzerland

  • A. Hackenberg

    2   Kinderuniversitätsspital Zürich, Neuropädiatrie, Zürich, Switzerland

  • N. Khan

    5   Kinderuniversitätsspital Zürich, Moyamoya Center, Zürich, Switzerland
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Background/Purpose: Moyamoya angiopathy (MMA) is a rare progressive cerebrovascular disorder marked by stenosis of cerebral arteries leading to reduced blood flow and risk of recurrent strokes. Moyamoya (MM) describes the tangled collaterals that develop in response to the reduced blood flow. Early diagnosis and intervention are crucial to managing symptoms and preventing further neurological damage. To date, no published data exist on brain volume in pediatric MMA.

Methods: Cerebral MRIs of children with MMA without stroke were analyzed. T1 pre-contrast sequences were segmented into defined compartments of gray and white matter measured in cm3 with the software “Freesurfer.” Volumes were compared between MM and healthy controls using a two-sample t-test and linear regression models. Volume size was correlated with a previously developed MM angiographic scoring system.

Results: A total of 19 patients with MMA (14 male) and 21 age- and sex-matched controls were included. Median duration of disease before MRI was 0.8 years. After adjusting for age and sex, there was a significant difference in the volume of the corpus callosum between MM patients and controls (p = 0.03). The callosal mid-posterior area was mostly affected. Focusing on population average effects, there was a significant reduction in total cortical gray matter (p = 0.009) and white matter volume (p = 0.006) in MM. For MMA affecting the posterior circulation (n = 38), there was evidence for decreased cortical gray and white matter volumes compared to MMA restricted to the anterior circulation. Duration of MM disease before MRI did not influence brain volumes.

Conclusion: MMA is a chronic condition characterized by a reduction in white and gray matter volume that precedes clinical ischemic events. This reduced brain volume is likely due to chronic underperfusion indicating that early revascularization treatment can provide clinical benefits.



Publication History

Article published online:
08 October 2024

Georg Thieme Verlag KG
Stuttgart · New York