Optic Nerve Shadow Enlargement in the Klippel-Trenaunay-Weber Syndrome

ABSTRACT A 10-year-old girl with Klippel-Trenaunay-Weber syndrome (KTWS) showed neuroradiology signs of enlarged optic nerve and medial rectus shadows. In 3 years of follow-up, her vision has remained normal. This is the first report of optic nerve enlargement in this syndrome. The case demonstrates that enlargement of the optic nerve shadows is not inevitably associated with visual deterioration. Optic nerve enlargement may be part of a hamartomatous process that involves other orbital contents.