Large vascular malformation in a child presenting with vascular steal phenomenon managed with pial synangiosis

Case report

Michael J. Ellis M.D. 1 , Derek Armstrong F.R.C.P.C. 2 and Peter B. Dirks M.D., Ph.D., F.R.C.S.C. 1
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  • 1 Divisions of Neurosurgery and
  • 2 Neuroradiology, Hospital for Sick Children, University of Toronto, Ontario, Canada
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The management of large and giant arteriovenous malformations (AVMs) in patients presenting with nonhemorrhagic neurological deficits secondary to vascular steal phenomenon is challenging and controversial. In many cases, large AVMs cannot be completely excised or cured, leaving patients with residual or partially treated AVMs, the natural history of which is unknown. Additionally, large, diffuse vascular malformations with multiple, small feeders, slow flow, or so-called cerebral proliferative angiopathy represent a related but distinct clinical and angiographic entity that may require a different therapeutic approach than traditional brain AVMs. The current management of children with other conditions of chronic cerebral hypoperfusion, such as moyamoya disease, involves consideration of surgical revascularization to enhance blood flow to the compromised hemisphere. Here, the authors present the case of a young child with a large thalamic vascular malformation who presented with clinical and radiological features of vascular steal and ischemia. In an effort to augment flow to the hypoperfused brain and protect against future ischemia, the authors treated the child with unilateral pial synangiosis. At 12 months, postoperative angiography demonstrated robust neovascularization, and the child has not sustained any further ischemic events. The authors discuss concept of vascular malformation–related hypoperfusion and the utility of indirect revascularization for inoperable vascular malformations presenting with ischemic symptoms.

Abbreviations used in this paper: AVM = arteriovenous malformation; CPA = cerebral proliferative angiopathy; ECA = external carotid artery; ICA = internal carotid artery; MCA = middle cerebral artery; MMA = middle meningeal artery.

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Contributor Notes

Address correspondence to: Peter B. Dirks, M.D., Ph.D., Hospital for Sick Children, Room 1503, 555 University Avenue, Toronto, Ontario, Canada M5G1X8. email: peter.dirks@sickkids.ca.
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