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

Radiology Cases of Sturge-Weber Syndrome

CT of Sturge-Weber syndrome
Axial CT with contrast of the brain shows diffuse leptomeningeal enhancement involving the right temporal and parietal and occipital lobes, an enlarged enhancing right choroid plexus, an enlarged medullary vein draining into the deep venous system, and some mild atrophy of the right cerebral hemisphere.