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

• Main Authors: S Podury, Bipasha Mukherjee
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2020-01-01
• Series: TNOA Journal of Ophthalmic Science and Research
• Subjects: congenital glaucoma; nevus of ota; phakomatosis pigmentovascularis; sturge–weber syndrome
• Online Access: http://www.tnoajosr.com/article.asp?issn=2589-4528;year=2020;volume=58;issue=4;spage=310;epage=312;aulast=Podury

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.