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Developmental Venous Anomaly (DVA)

Summary

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  • DVAs are congenital vascular malformations characterised by a radial arrangement of dilated medullary veins converging on a large collecting vein
  • Most common cerebral vascular malformation, typically asymptomatic and incidentally discovered on neuroimaging
  • Generally considered benign and do not require treatment, but may rarely be associated with other vascular malformations or haemorrhage1

Pathophysiology

  • Result from arrested development of venous system during embryogenesis
  • Represent persistent embryonic medullary veins that failed to regress
  • Function as normal drainage pathways for brain parenchyma
  • Typically drain into deep or superficial venous systems

Demographics

  • Prevalence: 2.6% in autopsy series, up to 6.4% in MRI studies
  • No significant gender predilection
  • Can occur at any age, but most commonly diagnosed in adults

Diagnosis

  • Usually asymptomatic and discovered incidentally on imaging
  • Rarely associated with:
    • Headaches
    • Seizures
    • Focal neurological deficits
  • May coexist with other vascular malformations (e.g., cavernous malformations)

Imaging

  • CT:
    • Contrast-enhanced: "caput medusae" appearance of converging veins
    • Non-contrast: may show calcifications or associated cavernomas
  • MRI:
    • T1-weighted: flow void of draining vein
    • T2-weighted: hypointense radial veins
    • Susceptibility-weighted imaging (SWI): prominent veins
    • Post-contrast T1: enhancement of radial veins and draining vein
  • Angiography:
    • "Caput medusae" appearance in venous phase
    • Normal arterial phase

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  • Incidental findings of a dilated vein with the caput medusa sign.
  • The DVA was associated with a small cavernoma (red arrow) .

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  • Incidental finding of a flow void on all sequences with a wider caput medusae on the SWI minimum intensity projection.

Treatment

  • Generally, no treatment required for asymptomatic DVAs
  • Management focuses on associated conditions:
    • Cavernous malformations: may require surgical resection
    • Intracranial haemorrhage: conservative management or surgical intervention based on severity
  • Anticoagulation should be used cautiously in patients with DVAs
  • Surgical resection of DVAs is contraindicated due to risk of venous infarction

Differential diagnosis

Imaging differential Differentiating feature
Cavernous malformation Popcorn lesion with haemosiderin rim; often coexists with a DVA
Arteriovenous malformation Arterial feeders and early venous drainage (shunt) rather than a purely venous phase
Capillary telangiectasia Brush-like SWI signal without a large collecting vein
Dural arteriovenous fistula Arteriovenous shunting with cortical venous reflux
Demyelination (central vein) A single central vein within a lesion, not a radial caput medusae

  1. Hsu et al. Symptomatic Developmental Venous Anomaly: State-of-the-Art Review on Genetics, Pathophysiology, and Imaging Approach to Diagnosis. 2023. AJNR. American journal of neuroradiology - Open in new tab. ↩