Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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February 22, 2018
High CBV due to Developmental Venous Anomaly (DVA)
- Background:
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Most frequently encountered cerebrovascular variation (~60% of all vascular anomalies)
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DVAs represent congenital enlargement of a cluster of medullary veins draining into a central collector and are commonly found draining the periventricular white matter.
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The estimated prevalence ranges from 0.6–2.5%.
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15–20% of patients have coexisting cavernomas or capillary malformations.
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- Clinical Presentation:
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Most are clinically silent and are detected incidentally on imaging or at autopsy.
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Their sizes vary from small, isolated lesions to holohemispheric or multifocal lesions, which may present with chronic venous congestion or hydrovenous disorders.
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In rare instances, may cause seizures (in setting of cortical dysplasia) or focal neurologic deficits (due to hemorrhage > thrombosis)
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Symptomatic DVAs have increasingly been identified presenting with a variety of neurologic symptoms, including paresthesias, motor deficits, trigeminal neuralgia, and extrapyramidal disorders.
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- Key Diagnostic Features:
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“Medusa head” appearance of dilated medullary white matter veins converging into a large “collector” vein, which drains into the dural sinuses or ependymal vein
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Resultant relative venous outflow restriction in regional cerebral parenchyma is illustrated on CT and MR perfusion images, showing increased CBV and MTT, resulting in an increased CBF.
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Perfusion techniques have contributed to a better understanding of the local hemodynamics related to uncomplicated DVAs.
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No correlation between perfusion parameters and the presence or type of symptoms has been properly established. Perfusion parameters are not able to predict complications related to DVAs.
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- Differential Diagnoses:
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Vascular neoplasm
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Chronic dural sinus thrombosis
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- Treatment:
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No therapy is required for solitary anomalies.
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Coexisting lesions are treated per standard of care.
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