Neovascularization precedes occlusion in moyamoya disease: angiographic findings in 172 pediatric patients

Eur Neurol. 2014;72(5-6):299-305. doi: 10.1159/000365286. Epub 2014 Oct 14.

Abstract

Background: Both basal collaterals (BC) and cortical microvascularization (CM) on angiography have been suggested as moyamoya disease (MMD)-specific findings; however, it is unknown whether the vascular network represents compensatory mechanisms for vascular occlusion or aberrant active neovascularization.

Methods: We investigated the grade of antegrade MCA flow, the degree of BC, and the presence of CM on conventional angiography in relation to disease severity in pediatric MMD. CM was defined as enlarged and winding distal cortical arteries and categorized into anterior or posterior CM depending on their sources. Findings from basal and acetazolamide stress brain perfusion SPECT studies were also evaluated.

Results: A total of 172 pediatric patients with MMD were enrolled in this study. As the severity of MMD increased, the grade of antegrade MCA flow gradually diminished. While the degree of BC peaked at Suzuki stage 3-4, CM was frequently observed at early MMD stages. About two-thirds of hemispheres with normal antegrade MCA flow on angiography and normal perfusion status on SPECT had anterior and/or posterior CM. Both anterior and posterior CM gradually decreased with the advancement of MMD.

Conclusion: Our findings from a large cohort of angiographically confirmed pediatric MMD patients indicate that neovascularization may occur before significant hemodynamic impairment in MMD.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acetazolamide
  • Brain / blood supply*
  • Brain / diagnostic imaging
  • Brain / pathology
  • Brain / physiopathology*
  • Cardiovascular Agents
  • Cerebral Angiography
  • Cerebrovascular Circulation / drug effects
  • Cerebrovascular Circulation / physiology
  • Child
  • Child, Preschool
  • Disease Progression
  • Female
  • Humans
  • Male
  • Microvessels / diagnostic imaging
  • Microvessels / pathology
  • Microvessels / physiopathology
  • Middle Cerebral Artery / diagnostic imaging
  • Middle Cerebral Artery / pathology
  • Middle Cerebral Artery / physiopathology
  • Moyamoya Disease / diagnostic imaging
  • Moyamoya Disease / pathology
  • Moyamoya Disease / physiopathology*
  • Neovascularization, Pathologic*
  • Retrospective Studies
  • Severity of Illness Index
  • Stress, Physiological
  • Tomography, Emission-Computed, Single-Photon

Substances

  • Cardiovascular Agents
  • Acetazolamide