FIGURE 13.

Conjunctival findings in patients with the Sturge-Weber syndrome. Dilatation and tortuosity of conjunctival and episcleral vessels become increasingly evident over time. Top (patient 18, Table), a background episcleral blue hue is evident in the left eye of 20-year-old. Conjunctival vessel tortuosity reveals frank corkscrewing, a sign pathognomonic for elevated transluminal venous pressure gradient. Slowed venous blood flow is also evidenced by boxcarring phenomenon noted on slit-lamp examination. Slight chemosis is present inferiorly, indicative of venous transudate taxing lymphatic drainage capacities. Bottom (patient 17, Table), Similar tell-tale corkscrewing of conjunctival vessels in another affected young adult with the Sturge-Weber syndrome and severe glaucoma.