Sturge-Weber syndrome

Discussion:

Sturge-Weber syndrome, or encephalotrigeminal angiomatosis, is a phacomatosis characterized by facial port wine stains and pial angiomas. 

CT findings:

  • detects subcortical calcification at an earlier age than plain film and can also demonstrate associated parenchymal volume loss
  • tram-track sign of cortical and subcortical calcification 1,2
  • calvarial and regional sinus enlargement may be evident
  • ipsilateral choroid plexus may be enlarged
  • in severe cases, a Dyke-Davidoff-Masson appearance may be seen
  • orbital choroidal hemangiomas may be present
  • asymmetric cavernous sinus enlargement 3
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