Sturge-Weber syndrome

Case contributed by Sepehr Haghighi


Recurrent seizure

Patient Data

Age: 4 years
Gender: Female

In scanogram, evidence of gyriform calcification is detected in central part of skull from anterior to posterior.

In non-contrast brain CT scan, unilateral right-sided atrophy of cerebral hemisphere with gyriform cortico-subcortical (tram-track) calcification are seen. Increased subarachnoid space on right side and mild thickening of overlying calvarium is seen. No ventricular dilation or obvious choroid plexus enlargement is noted.

Also, cortical calcification at the right cerebellar hemisphere is visible without sign of atrophy.

These findings are consistent with Sturge-Weber syndrome.

Case 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

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.

Updating… Please wait.

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

 Thank you for updating your details.