June 3, 2010

Sturge-Weber Syndrome

Axial CT images show "railroad track" calcifications (arrows) in the left occipital cortex with ipsilateral enlargement of the choroid plexus (arrowhead) in this patient with a port-wine stain in the left V1 distribution.

  • Also known as encephalotrigeminal angiomatosis
  • Sporadic disorder affecting skin and central nervous system
  • Defined as capillary malformation of the leptomeninges with or without choroid and facial V1 or V1-V2 involvement (port-wine stain)
  • Probably due to embryonic defect of persistent vascular plexus in the neural tube during 6th week of embryonic development
  • Port-wine stains can be unilateral or bilateral, most commonly involve V1 distribution but can also be extracranial
  • Intracranial involvement always ipsilateral to the port-wine stain of the face, occipital lobe most common
  • MRI more sensitive than CT in identifying secondary changes due to leptomeningeal capillary malformation
  • Cerebral cortical atrophy, compensatory ventricular and choroid plexus enlargement, calvarial hemihypertrophy and superficial gyriform enhancement after gadolinium injection
  • "Railroad track" calcification of the cerebral cortex caused by precipitation of calcium likely due to alternation of vascular dynamics of the leptomeningeal malformation

Gorlin RJ, Cohen MM, Hennekam RCM. Syndromes of the Head and Neck, 4th ed, 2001.
Muller-Forell WS. Imaging of Orbital and Visual Pathway Pathology, 2005.

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