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.

Facts

• 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

Imaging

• 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

Reference:

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.