Sturge-Weber Syndrome

  • Etiology: Phakomatosis which is venous malformation
  • Imaging:
    — Facial venocapillary malformation (port wine nevi or stain) in cranial nerve V1 distribution
    — Leptomeningeal venous angiomatosis = occlusion of surface veins and superior sagittal sinus leads to ischemia and atrophy and gyriform calcification (tram track) of cortex
    — Dilated medullary vein draining into dilated deep vein
    — Enlarged choroid plexus
    — Calvarial thickening
    — Opthalmologic findings: Buphthalmos due to obstruction and elevated venous pressures
  • Imaging CT:
    — Tram track cortical calcification and hemiatrophy of affected lobe
    — Enlargment of choroid plexus in lateral ventricles
  • Imaging MRI:
    — May have abnormal ocular enhancement from choroidal angioma or inflammation from glaucoma
    — T1WI post contrast: Dramatic leptomeningeal enhancement which more fully demonstrates the degree of vascular abnormality than CT, can have cortical enhancment from ischemic injury
  • DDX:
  • Complications:
  • Treatment: Does not spontaneously regress and does not respond to propranolol or steroids
  • Clinical:
    — Port-wine stain in V1 distribution
    — Seizures or dementia or hemiplegia
    — Hemianopsia or bupthalmos or glaucoma
    — Possible scleral and choroidal angiomata
    — Possible associated angio-osteohypertrophy of Klippel-Tenaunay syndrome
    — Present at birth and grows in proportion with child
    — 80% is unilateral and 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.
MRI of Sturge-Weber syndrome
Axial (above left), coronal (above right) and sagittal (below) T1 MRI with contrast of the brain shows left-sided diffuse, marked leptomeningeal enhancement. There is mild cortical atrophy of the left cerebral hemisphere.

Clinical Cases of Sturge-Weber Syndrome

Clinical image of Sturge-Weber Syndrome
Clinical image shows a left-sided port-wine stain in the V1 distribution of the fifth cranial nerve.
Clinical image of Sturge-Weber Syndrome
Clinical image shows a left-sided port-wine stain in the V1 distribution of the fifth cranial nerve.