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

Abstract

<jats:p>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.</jats:p>

Encephaloduroarteriosynangiosis for cerebral proliferative angiopathy with cerebral ischemia

“…There have been several case reports on patients with CPA, [1][2][3][4][5][6][7][8][9]11,12,15 and one series of 49 patients with this disorder. 10 In that study of 49 patients (mean age 22 years), 45% presented with seizures, 41% with severe and often disabling headaches, 12% with hemorrhage, and 16% with nonhemorrhagic neurological deficits.…”

“…Treatments for CPA are challenging and controversial, 10 and there are only a few detailed reports on the treatment of CPA with cerebral ischemia. 4,12 We report the first case of adult CPA with cerebral ischemia successfully treated by encephaloduroarteriosynangiosis (EDAS).…”

“…4,12 In one case, a 2-year-old girl with CPA suffered from an acute infarct and was treated with pial synangiosis. 4 Although no imaging studies for cerebral perfusion were performed, the authors considered that the patient had impaired cerebrovascular reserve. To prevent future ischemic events, the patient underwent pial synangiosis.…”

Cerebral revascularization in cerebral proliferative angiopathy: a systematic review

“…All studies utilized indirect forms of revascularization with 3 patients receiving calvarial burr holes, 18,26 and the remaining 4 receiving various forms of encephaloduroarteriosynangiosis (EDAS). 4,15,19,25 No studies utilized direct revascularization.…”

Brain Reorganization after Endovascular Treatment in a Patient with a Large Arteriovenous Malformation: The Role of Diagnostic and Functional Neuroimaging Techniques

“…the so-called "steal phenomenon" -described as both the relative hypoperfusion due to shunting of blood and decreased perfusion pressure caused by venous hypertension in the brain tissue adjacent to the avM area -has been considered a possible cause of neurological symptoms 12 . despite evidence provided by neuropsychological studies following treatment 13 and by PEt studies before and after endovascular procedures 14,15 , the debate on the significance of the "steal phenomenon" in avMs remains open and a consensus has not been achieved in the literature.…”