Conjunctival melanosis with retinal vessel tortuosity: An unusual presentation of Sturge–Weber syndrome

Abstract

Sturge–Weber syndrome (SWS) is a rare neuro-oculocutaneous disorder. The classical signs are unilateral facial port wine stain, leptomeningeal angiomas, and their sequele with ipsilateral glaucoma. Here, we are reporting two cases of SWS associated with conjunctival melanosis and retinal vascular tortuosity. This unusual coincidence maybe representative of a unique entity named phacomatosis pigmentovascularis.

Keywords

• Conjunctival melanosis
• leptomeningeal angioma
• phacomatosis pigmentovascularis
• port wine stain
• retinal vessel tortuosity
• Sturge–Weber syndrome