Go Back Brain/13. Brain and meninges, Supratentorial/Normal, Technique, Anomaly/13.1 - Sturge Weber Syndrome/ Go to Index

Precise etiology uncertain

Port-wine stain in 5th nerve distribution

Believed to result from failure of development of normal cortical venous drainage with persistence of primitive vascular plexus

Pial vascular malformation develops with thin-walled, dilated capillaries and venules.

Pial angiomatous malformation usually ipsilateral to port-wine nevus on the face.

Usually affects posterior cerebral hemisphere (occipital lobe most commonly followed by parietal and temporal lobes).

Imaging

“Tram-track” calcification representing calcification of opposing gyri.

Contrast enhanced MRI demonstrates enhancing pial angiomatous malformation.

Collateral venous drainage is manifested by increase in size and number of medullary and subependymal veins

Hypertrophy of ipsilateral choroid plexus.

Increased T2 signal in ipsilateral white matter secondary to gliosis and demyelination.

Secondary changes include compensatory hypertrophy of ipsilateral diploic space and enlargement of ipsilateral paranasal sinuses and mastoid air cells.