Macular vasculopathy and its evolution in incontinentia pigmenti.

Objective: To describe macular vasculopathy in incontinentia pigmenti.

Methods: Twelve baby girls with incontinentia pigmenti were examined under general anesthesia by fluorescein angiography of the macula. Nine eyes of 9 patients had sufficient detail to allow evaluation of capillary changes. Angiography was initiated as early as 3 months of age and was repeated in 7 eyes at 3- to 12-month intervals. Changes in capillary patterns were identified.

Results: Irregularly enlarged or distorted foveal avascular zones were noted in all 9 maculas. Sparseness of the perifoveolar capillary bed was a characteristic finding. Sequential macular angiography demonstrated non-progressive (stable) capillary closure in 2 eyes; progressive closure in another macula; progressive closure plus addition or reopening of macular capillaries in 3 eyes; and central retinal artery occlusion, with cherry-red spot formation, in 1 eye at 12 days of age. In addition, progressive tractional detachment of the macula occurred in 2 of these eyes, and progressive macular neovascularization occurred in 1 eye.

Conclusions: Macular ischemia is characteristic of incontinentia pigmenti and is often progressive. It is the initiating event of a typical vasculopathy, characterized by capillary remodelling and, occasionally, by neovascularization and tractional detachment of the retina.