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Year : 2020  |  Volume : 58  |  Issue : 4  |  Page : 310-312

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

Orbit, Oculoplasty, Reconstructive & Aesthetic Services, Sankara Nethralaya, Medical Research Foundation, Chennai, Tamil Nadu, India

Correspondence Address:
Dr. Bipasha Mukherjee
Sankara Nethralaya, Medical Research Foundation, 18, College Road, Chennai - 600 006, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_59_20

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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.

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