| CASE REPORT |
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| Year : 2020 | Volume
: 58
| Issue : 4 | Page : 310-312 |
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Double whammy: Congenital glaucoma in phakomatosis pigmentovascularis with Sturge–Weber syndrome and nevus of Ota
S Podury, Bipasha Mukherjee
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
India
 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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