Sturge-Weber syndrome

Case contributed by Abdallah Al Khateeb

Presentation

Withheld.

Patient Data

Age: 7 years
Gender: Male
mri
  • abnormal low T2 signal at the subcortical white matter on the right side, corresponding to the calcifications seen on CT scan.

  • prominent abnormal vascular channels traversing the white matter of the right hemisphere, along with abnormally enlarged signal void deep venous drainage.

ct

Few areas of faint calcification at the subcortical white matter of the right frontal, parietal and occipital lobes. No similar findings on the left side.

10 months later

mri
  • abnormal low T2 signal at the subcortical white matter on the right side, corresponding to the calcifications seen on CT scan.

  • prominent abnormal vascular channels traversing the white matter of the right hemisphere, along with abnormally enlarged signal void deep venous drainage.

  • mild volume loss of the right cerebrum.

  • diffuse leptomeningeal enhancement of the right hemisphere.

  • enlarged right side choroid plexus.

Case Discussion

This boy first presentation was with a right side face port wine stain at the trigeminal nerve V1 dermatome, which mandated further evaluation for the possibility of Sturge-Weber syndrome.

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