Abstract

Aims/Purpose: To present the angiographic findings in a case of a 6‐year‐old girl with incontinentia pigmenti.Methods: We describe the case of a 6‐year‐old girl with a known diagnosis of incontinentia pigmenti that presented with retinal vascular changes in a routine ophthalmologic evaluation. Fundoscopic examination revealed a peripheral avascular area with adjacent vascular proliferation. In order to better evaluate these findings, a fluorescein angiography was performed.Results: Angiography findings included abnormal vascular loops, capillary nonperfusion areas and vascular anastomoses. There were also leakage areas (corresponding to active neovascularization) and areas of staining (due to fibrosis). Laser photocoagulation of the peripheral avascular area was done.Conclusions: Incontinentia pigmenti often presents with neovascularization and other vascular changes that are more pronounced in the retinal periphery. Therefore, fluorescein angiography is very valuable in the documentation and study of vascular lesions associated with this condition.

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