Sturge-Weber Syndrome

A case of Sturge-Weber syndrome (Encephalotrigeminal angiomatosis) with angiomas that involve the leptomeninges, and the skin of the ipsilateral hemiface, associated with congenital glaucoma in the same eye. Various illustrations are included to demonstrate the port wine stain, enlarged optic nerve cupping (glaucomatous optic neuropathy), and typical MRI findings which may be seen in Sturge-Weber syndrome. Figure 1 : External photograph of the face of Sturge-Weber syndrome's patient demonstrating a Port wine stain on the right hemiface involving the forehead and upper eyelid. Figure 2 : Disc photo of the right eye demonstrating a large cup-to-disc ratio consistent with known glaucoma Figure 3 : Disc photo of the left eye demonstrating a normal optic nerve with small cup-to-disc ratio Figure 4 : Post-contrast T1-weighted axial MRI with fat suppression showing the right cerebral atrophy, associated with thickened adjacent calvarium Figure 5 : Post-contrast T1-weighted axial MRIVBsturgeweber, EECneurocutaneoussyndromessturgewebe