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Developmental venous anomaly: cerebellar atrophy and dystrophic calcifications

Case contributed by Bruno Di Muzio
Diagnosis almost certain

Presentation

Incidental finding on scan performed in the context of trauma.

Patient Data

Age: 20 years
Gender: Male

The posterior fossa is asymmetric, smaller and consequently distorted on the left. The left cerebellar hemisphere is smaller than the right and shows gross irregular calcifications, likely cortical/peripheral in distribution. No traumatic injuries identified. 

Disproportionate atrophy of the left cerebellar hemisphere with concordant asymmetry of the bony posterior fossa suggesting this is a congenital/long-term process. There are no features to indicate cerebellar dysplasia, with the cerebellar folia and the grey-white matter differentiation preserved. There is a left cerebellar developmental venous anomaly (best appreciated on susceptibility weighted sequences) draining anteriorly to veins in the left cerebellopontine angle. This is associated with multiple linear and nodular foci of susceptibility related signal loss, corresponding to foci of calcification demonstrated on recent CT. No T2 or FLAIR signal abnormality in the adjacent brain parenchyma. No evidence of recent hemorrhage.

Appearance and intensity of brain parenchyma are otherwise normal. Ventricular system and cisternal spaces appear normal. MRA time-of-flight is unremarkable, with no other vascular malformations identified. 

Case Discussion

This case illustrates a left cerebellar DVA associated with locoregional atrophy and likely dystrophic calcification. No convincing evidence of associated cavernoma, although a tiny cavernoma cannot necessarily be excluded.

It has been described that local venous congestion may account for the parenchymal abnormalities, such as the ones demonstrated in this case, related to DVAs. Studies have shown that commonly associated abnormalities include 1:

  • atrophy (~30%)
  • white matter lesions (~30%)
  • dystrophic calcifications (~10%)

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