Double whammy: Congenital glaucoma in phakomatosis pigmentovascularis with Sturge–Weber syndrome and nevus of Ota

Abstract

Phakomatosis pigmentovascularis (PPV) is a rare congenital condition characterized by a combination of cutaneous capillary hemangiomas and dermal melanocytosis. The dual presentation of Sturge–Weber syndrome (SWS) and nevus of Ota in the same patient is a rare finding. Individually, both these conditions can predispose to developmental glaucoma. The combined presentation of these two predisposing conditions has a very high chance of coexisting glaucoma. We report a patient of PPV presenting with bilateral SWS, nevus of Ota, and congenital glaucoma.

Keywords

• congenital glaucoma
• nevus of ota
• phakomatosis pigmentovascularis
• sturge–weber syndrome