Developmental venous anomaly

Dr Owen Kang and Dr Donna D'Souza et al.

Developmental venous anomaly (DVA), also known as cerebral venous angioma, is a congenital malformation of veins which drain normal brain. They were thought to be rare before cross-sectional imaging but are now recognised as being the most common cerebral vascular malformation, accounting for ~55% of all such lesions.

A DVA is characterised by the caput medusae sign of veins draining into a single larger collecting vein, which in turn drains into either a dural sinus or into a deep ependymal vein. The appearance has also been likened to a palm tree.

Developmental venous anomalies are usually incidental findings. However, patients can present with intracranial haemorrhage (1-5%). An association has also been described with ischaemic stroke and epilepsy 8

The aetiology of developmental venous anomalies remains uncertain but may relate to arrested development of venous structures 2-3. Histologically they consist of a number of abnormally thickened veins with normal feeding arteries and capillaries 3.

The most common locations are:

However, DVAs can be seen anywhere, draining either superficially or deep.

Developmental venous anomalies are seen on both CT and MRI as a leash of vessels draining towards a central vein.

If large, the draining vein may be seen on non-contrast CT and is confirmed with contrast administration as a linear or curvilinear enhancing structure.

Dystrophic calcifications may be seen associated in up to 30% of the cases 9. This can be particularly prominent in the basal ganglia and thalami resulting in unilateral calcification 10,11.

Angiographically the caput medusae appearance (collection of dilated medullary veins converge in an enlarged transcortical or subependymal collector vein) is pathognomonic and seen only in the venous phase. Arterial phase appears normal although late capillary blush may be present. No shunting is present.

Developmental venous anomalies are often visible on most sequences but can be subtle and are most easily seen on postcontrast T1 sequences and susceptibility weighted imaging (SWI). If there is an associated cavernous haemangioma, then susceptibility weighted sequences will be most sensitive to this component.

Additionally, in ~10% of cases, high T2 signal will be seen in the surrounding white matter; this may be due to gliosis, oedema or leukoaraiosis 12.

SWI is the preferred sequence in venous anomalies and proved to have better detectability of venous structures than conventional T2*-weighted imaging. Signals on SWI are not compromised by low-velocity venous flow. Therefore, SWI has successfully demonstrated low-flow vascular formations such as DVA. The signal intensity of veins will be low on SWI images but will vary on phase imaging depending on the vendor. It will, however, be the same as other veins and the opposite of calcification.

If isolated developmental venous anomalies require no treatment. If part of a mixed vascular malformation then treatment will be predicated on the other component. Informing the surgeon of the presence of a DVA is, however, essential as cautery of the collecting vein can lead to venous infarction of the brain parenchyma it drains.

When isolated, developmental venous anomalies have a very low complication rate (0.15% per annum) mainly from spontaneous thrombosis of the collecting vein leading to venous infarction and haemorrhage.

A more recent study has demonstrated that in exceedingly rare cases, the DVA can become symptomatic by various vascular complications. The authors suggested identification of the underlying pathomechanism by MR and DSA for proper management. The importance of the preserved integrity of the DVA itself still holds true 6.

Generally, the appearances will be typical and no differential should be offered. In some instances, imaging appearances may be atypical or be confounded by concurrent pathology (e.g. haemorrhage). In such cases it is worth considering:

  • think of an associated cavernoma when a developmental venous anomaly is found in the context of an intraparenchymal haemorrhage investigation, as isolated DVAs rarely bleed
  • developmental venous anomalies can be subtle on many MRI sequences; T1 C+ and SWI are most sensitive
Share article

Article information

rID: 1215
Synonyms or Alternate Spellings:
  • Developmental venous anomaly (DVA)
  • DVA
  • Cerebral venous angioma
  • Cerebral venous angiomas
  • Developmental venous anomalies
  • Developmental venous anomalies (DVA's)

Support Radiopaedia and see fewer ads

Cases and figures

  • Drag
    T1WI axial with Gad
    Case 1: mixed vascular malformation
    Drag here to reorder.
  • Drag
    Case 2: SWI
    Drag here to reorder.
  • Drag
    Case 3: SWI
    Drag here to reorder.
  • Drag
    Case 4: pontine DVA on CT
    Drag here to reorder.
  • Drag
    Angiogram of DVA
    Case 5: angiogram of DVA
    Drag here to reorder.
  • Drag
    Case 6: cerebellar haemorrhage from DVA
    Drag here to reorder.
  • Drag
    Case 7: SWI
    Drag here to reorder.
  • Drag
    Case 8
    Drag here to reorder.
  • Drag
    Case 9
    Drag here to reorder.
  • Drag
    Case 10: mixed vascular malformation
    Drag here to reorder.
  • Drag
    Case 11
    Drag here to reorder.
  • Drag
    Case 12: SWI
    Drag here to reorder.
  • Drag
    Case 13: cerebellar
    Drag here to reorder.
  • Drag
    Case 14: with cerebellar atrophy and dystrophic calcifications
    Drag here to reorder.
  • Drag
    Case 15: calcification of basal ganglia
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.