Sturge-Weber Syndrome

  • Etiology: venous malformation
  • Imaging:
    — Facial venocapillary malformation (port wine nevi/stain) in cranial nerve V1 distribution
    — Leptomeningeal angiomatosis = occlusion of surface veins + superior sagittal sinus -> ischemia + atrophy + gyriform calcification (tram track) of cortex
    — Dilated medullary vein draining into dilated deep vein, enlarged choroid plexus, calvarial thickening
  • Clinical: present at birth, grows in proportion with child, does not spontaneously regress, does not respond to propranolol / steroids, 80% is unilateral + 20% is bilateral

Cases of Sturge-Weber Syndrome